Classificazione,inquadramento diagnostico e trattamento della lesione di Monteggia in età pediatrica

LO SCALPELLO-OTODI Educational - Monteggia fractures represent the association of a fracture of the proximal ulna and luxation of the radial head. This type of lesion is infrequent and may be...     

Anti-inflammatory Effects of Novel P2X4 Receptor Antagonists,NC-2600 and NP-1815-PX,in a Murine Model of Colitis

Inflammation - The pharmacological blockade of P2X4 receptors has shown potential benefits in the management of several immune/inflammatory diseases. However, data regarding the involvement of P2X4...     

The immune development in a child born to a cyclosporin A-treated woman with systemic lupus erythematosus/polymyositis

The case of a woman affected by an overlap syndrome systemic lupus erythematosus/polymyositis (PM), who presented with active myositis at the start of the pregnancy, is reported. Therapy with cyclosporin, corticosteroids, hydroxychloroquine and high-dose intravenous immunoglobulin induced a progressive remission of clinical and laboratory signs of myositis. At 33 weeks of gestation, after a premature pre-term rupture of membrane, a male child was delivered. Although premature, and small for gestational age, he had a normal growth, and did not show any clinical sign suggestive of immune deficiency. Lymphocyte phenotypical and functional studies, as well as response to vaccination, were also normal.     

Clinical effects of oral theophylline in sick sinus syndrome

In 17 patients with symptomatic sinus bradycardia (age: 66 +/- 11 years), a resting electrocardiogram, a 24-hour Holter recording, and a treadmill test were performed both before and after administration of slow-release theophylline (700 mg daily). The drug increased resting heart rate (46 +/- 7 versus 62 +/- 18 beats/min, p less than 0.01), mean 24-hour rate (51 +/- 6 versus 64 +/- 16 beats/min, p less than 0.01), and minimal 24-hour heart rate (36 +/- 6 versus 43 +/- 10 beats/min, p less than 0.05). Cardiac pauses greater than 2.5 seconds were present in four patients during control recording, and disappeared after theophylline. The daily number of premature supraventricular and ventricular beats increased slightly after the drug. Exercise heart rate was higher after theophylline than during the control test (p less than 0.01). Thirteen patients were followed for a period of 17 +/- 3 months. Suppression of symptoms was achieved in 12 patients. Asthenia and easy fatigue were reduced markedly by the drug. During long-term therapy, the sinus rate was similar to that observed at the steady-state evaluation. In 3 of the 17 patients theophylline had to be discontinued because of gastric intolerance (in two at the end of the steady-state evaluation and in one during long-term therapy). These data suggest that oral theophylline can represent an effective therapy in some patients with symptomatic sinus bradycardia.     

Thrombocytosis and leukocytosis interaction in vascular complications of essential thrombocythemia

To elucidate the role of thrombocytosis, alone or in combination with standard (age, previous cardiovascular events) and novel (leukocytosis, JAK2(V617F) mutational status) risk factors, in the cardiovascular events of essential thrombocythemia (ET), we analyzed a cohort of 1063 patients. We found that a platelet count at diagnosis greater than 1000 x 10(9)/L was associated with significantly lower rate of thrombosis in multivariable analysis and, if combined with leukocytes less than 11 x 10(9)/L, pointed to a "low-risk" category with a rate of thrombosis of 1.59% of patients/year. On the contrary, the highest risk category (thrombosis rate, 2.95% of patients/year) was constituted of patients with leukocytosis, lower platelet count, and a JAK2(V617F) mutated genotype in most cases (77% vs 26% in the low-risk group), independently from standard risk factors. These data challenge the theory that elevated platelet count increases thrombosis risk in ET and suggest prospective clinical trials to support this hypothesis.     

Anaemia characterises patients with myelofibrosis harbouring Mpl mutation

The clinical and haematological phenotype of patients with myelofibrosis harbouring MPL(W515L/K) mutation has not been thoroughly investigated. Of 217 myelofibrosis subjects, 18 (8.2%) had an MPL mutation, four of which (22%) co-existed with JAK2(V617F) mutation. When compared with MPL wild-type patients, irrespective of JAK2(V617F) status, those with MPL(W515L/K), were more frequently female, were older (61 years vs. 57 years; P = 0.02), presented with more severe anaemia (haemoglobin, 101 g/l vs. 121 g/l; P = 0.002) and were more likely to require regular transfusional support (P = 0.012). These data indicate that MPL mutation in myelofibrosis characterises patients with more severe anaemic phenotype.     

Coronary artery fistula: diagnostic role of transthoracic and transesophageal echocardiography

Congenital coronary artery fistulas (CAFs) are rare. Some patients develop symptoms of congestive heart failure secondary to a large left-to-right shunt or myocardial ischemia from coronary artery steal in the first few years after birth. After the second decade the frequency of symptoms and complications increase. We report a case of CAF originating from the circumflex artery and draining into the coronary sinus, associated with left main coronary aneurysm. Transtho-racic and transesophageal echocardiography approach showed the origin, course, and drainage site of the CAF. This case represents a typical sample of this rare anomaly and puts into evidence the essential role of echocardiography to define and complete the angiographic diagnosis.     

An individualized rehabilitation program in patients with systemic sclerosis may improve quality of life and hand mobility

Few data are available to assess the efficacy of rehabilitative interventions in systemic sclerosis (SSc). We refer here the results of an individualized rehabilitation program in 16 patients with SSc. In particular, when possible, the number of patients who achieved a minimal clinically important difference (MCID) was determined. Results were evaluated taking advantage of the development of validated questionnaires and tests to assess quality of life (QOL) and disability in SSc. At the end of a period of 4 months of observation, 69% and 62% of patients reported an improvement of the physical and mental components of the SF-36 higher than the MCID (as established in other rheumatic conditions). Analogously, an improvement of the impact of respiratory disease on patients’ QOL, as assessed by the Saint George’s Respiratory Questionnaire, was perceived by 67% of them. These results might be explained by better exercise tolerance, which was suggested by the significant reduction of the heart rate and of a visual analogue scale for dyspnoea at the end of the 6-min walking test. Finally, a statistically significant improvement of hand mobility, as assessed by the hand mobility in scleroderma test was obtained. This study suggests that a significant proportion of patients with SSc experience an improvement in their perception of QOL, a better exercise tolerance, and a better hand mobility after a rehabilitation program consisting by a 2-week period of daily individual 30-min sessions as outpatient, followed by at-home exercise program.     

RGD‐mimic polyamidoamine–montmorillonite composites with tunable stiffness as scaffolds for bone tissue‐engineering applications

This paper reports on the development of montmorillonite (MMT)‐reinforced hydrogels, based on a peptidomimetic polyamidoamine carrying guanidine pendants (AGMA1), as substrates for the osteo‐induction of osteoblast precursor cells. AGMA1 hydrogels of various degrees of crosslinking responded favourably to MMT reinforcement, giving rise to composite hydrogels with shear storage modulus G′, when fully swollen in water, up to 200 kPa, i.e. 20 times higher than the virgin hydrogels and of the same order or higher than other hydrogel‐based composites proposed for orthopaedic applications. This significant improvement was ascribed to the effective interpenetration between the polymer matrix and the inorganic filler. AGMA1–MMT hydrogels, when evaluated as scaffolds for the osteogenic differentiation of mouse calvaria‐derived pre‐osteoblastic MC3T3‐E1 cells, proved able to support cell adhesion and proliferation and clearly induced differentiation towards the osteoblastic phenotype, as indicated by different markers. In addition, AGMA1–MMT hydrogels proved completely degradable in aqueous media at pH 7.4 and did not provide any evidence of cytotoxicity. The experimental evidence suggests that AGMA1–MMT composites definitely warrant potential as scaffolds for osteoblast culture and bone grafts. Copyright © 2016 John Wiley & Sons, Ltd.