Acute chest syndrome in children with sickle cell disease: Data from a national AIEOP cohort identify priority areas of intervention in a hub-and-spoke system.

Acute chest syndrome (ACS) is a frequent cause of hospitalization in sickle cell disease (SCD). Despite advances in acute care, many settings still lack knowledge about ACS best practices. After the AIEOP Guidelines were published in 2012, suggesting standardized management in Italy, a retrospective study was performed to assess the diagnostic and therapeutic pathways of ACS in children. From 2013 to 2018, 208 ACS episodes were presented by 122/583 kids in 11 centres. 73 were male, mean age 10.9 years, 85% African, 92% HbSS or Sß°. In our hub-and-spoke system, a good adherence to Guidelines was documented, but discrepancies between reference centres and general hospitals were noted. Improvement is needed for timely transfer to reference centres, use of incentive spirometry, oxygen therapy and pain management.

A management perspective on resilience in healthcare: a framework and avenues for future research.

Recent major health shocks, such as the 2014-16 Ebola, the Zika outbreak, and, last but not least, the COVID-19 pandemic, have strongly contributed to drawing attention to the issue of resilience in the healthcare domain. Nevertheless, the scientific literature appears fragmented, creating difficulties in developing incremental research in this relevant managerial field.To fill this gap, this systematic literature review aims to provide a clear state of the art of the literature dealing with resilience in healthcare. Specifically, from the analysis of the theoretical articles and reviews, the key dimensions of resilience are identified, and a novel classification framework is proposed. The classification framework is then used to systematize extant empirical contributions. Two main dimensions of resilience are identified: the approach to resilience (reactive vs. proactive) and the type of crisis to deal with (acute shocks vs. chronic stressors). Four main streams of research are thus identified: (i) proactive approaches to acute shocks; (ii) proactive approaches to chronic stressors; (iii) reactive approaches to acute shocks; and (iv) reactive approaches to chronic stressors. These are scrutinised considering three additional dimensions: the level of analysis, the resources to nurture resilience, and the country context. The classification framework and the associated mapping contribute to systematising the fragmented literature on resilience in healthcare, providing a clear picture of the state of the art in this field and drawing a research agenda that opens interesting paths for future research.

Phantom study of stereotactic radioablation for ventricular tachycardia (STRA-MI-VT) using Cyberknife Synchrony Respiratory Tracking System with a single fiducial marker.

PURPOSE: Within the STRA-MI-VT phase Ib/II trial (NCT04066517), the aim of this phantom study was to explore the feasibility of Cyberknife treatments on cardiac lesions by tracking as a single marker the lead tip of an implantable cardioverter defibrillator. The residual displacement of the lesion during the tracking was studied, planning margins were found and the dosimetric accuracy of the treatment was checked. MATERIALS AND METHODS: A lead was inserted into a phantom (EasyCube phantom, Sun Nuclear Co, USA) and then placed on the translating ExacTrac Gating System (BrainLAB AG, Germany). The phantom was rotated, a virtual lesion was identified and its displacement during the tracking was studied. Two plans were compared, calculated on the unrotated volume and on the envelope of the unrotated and the rotated volumes. The plans were delivered using the Cyberknife System (Accuray Inc, USA) and their dosimetric accuracy verified by gamma analysis with gafchromic films. RESULTS: The residual margin increases enhancing the distance between the lead and the lesion. It is 4 mm for distance 0 cm and 5 mm for distance 5 cm. The coverage is reduced by 3.8% (interquartile range 2.5%-4.7%) when the dose is prescribed on the unrotated volume. All treatment plans are accurate and 3% 3 mm gamma analysis results are greater than 94%. CONCLUSIONS: Results showed that tracking with a single marker is feasible considering adequate residual planning margins. The volumes could be further reduced by using additional markers, for example by placing them on the patient's skin.

STRA-MI-VT (STereotactic RadioAblation by Multimodal Imaging for Ventricular Tachycardia): rationale and design of an Italian experimental prospective study.

BACKGROUND: Ventricular tachycardia (VT) is a life-threatening condition, which usually implies the need of an implantable cardioverter defibrillator in combination with antiarrhythmic drugs and catheter ablation. Stereotactic body radiotherapy (SBRT) represents a common form of therapy in oncology, which has emerged as a well-tolerated and promising alternative option for the treatment of refractory VT in patients with structural heart disease. OBJECTIVE: In the STRA-MI-VT trial, we will investigate as primary endpoints safety and efficacy of SBRT for the treatment of recurrent VT in patients not eligible for catheter ablation. Secondary aim will be to evaluate SBRT effects on global mortality, changes in heart function, and in the quality of life during follow-up. METHODS: This is a spontaneous, prospective, experimental (phase Ib/II), open-label study (NCT04066517); 15 patients with structural heart disease and intractable VT will be enrolled within a 2-year period. Advanced multimodal cardiac imaging preceding chest CT-simulation will serve to elaborate the treatment plan on different linear accelerators with target and organs-at-risk definition. SBRT will consist in a single radioablation session of 25 Gy. Follow-up will last up to 12 months. CONCLUSIONS: We test the hypothesis that SBRT reduces the VT burden in a safe and effective way, leading to an improvement in quality of life and survival. If the results will be favorable, radioablation will turn into a potential alternative option for selected patients with an indication to VT ablation, based on the opportunity to treat ventricular arrhythmogenic substrates in a convenient and less-invasive manner.

Pediatric musculoskeletal injuries: role of ultrasound and magnetic resonance imaging.

Pediatric musculoskeletal system is particularly prone to traumatic and sports-related injuries, both acute and chronic, i.e., overuse injuries, because of inherent conditions of weakness, such as the open physis, representing the weakest aspect within the bone, the ligamentous supports and changing biomechanics. Being aware that a quick diagnosis is essential to preserve the good functionality of the limb involved, it is mandatory for the radiologist to recognize the most common patterns of these injuries, identifying those requiring a prompt surgery, as well as be confident with the technique performed, and be supported by an important background experience and knowledge skills.

The coexistence of primary hyperparathyroidism and thyroid nodules: should the preoperative work-up of the parathyroid and the thyroid diseases be specifically adjusted?

INTRODUCTION AND OBJECTIVES: Primary hyperparathyroidism (PHPT) can be found in concomitance with thyroid disease (TD) in a high frequency of cases. In this context the diagnostic exams for localizing the enlarged parathyroid(s) gland(s) could be less reliable or nonconclusive. Moreover, the thyroid carcinoma seems to be more frequent compared to that isolated thyroid desease and, therefore, carefully investigated. The main goal of the present study is to evaluate which diagnostic tool (US, MIBI) is more reliable for localizing the site of the PTH hypersecretion and to confirm if it is always advantageous a combination of both exams. Besides, we evaluated the incidence of thyroid carcinoma in our series of patients. PATIENTS AND METHODS: A review of available data of 73 patients who underwent total thyroidectomy + parathyroidectomy from 2003 and 2014 was performed. The preoperative workup included systematically US and MIBI whose results were considered true positive when at least the side (left/right) of the parathyroid affected were concordant with the surgical report, settled as the gold standard, according to the Cox nonnested model. The connection between the diagnostic results of US versus MIBI was calculated with the Cohen K index for evaluating their overlap. The average of the thyroid carcinoma were also calculated. RESULTS: The difference between respectively US versus surgical report (p value=0.73) and MIBI versus surgical report (p value=0.81) were not significant. The low Cohen K index showed that both US and MIBI are complementary. In 23 patients (32,9%) a thyroid carcinoma was found. CONCLUSIONS: The association of MIBI and neck US is mandatory in the first evaluation of patients undergoing thyroidectomy and parathyroid excision simultaneously. The high prevalence of thyroid carcinoma in this specific context suggests a more aggressive diagnostic and surgical behaviour.

Diagnostic value of computed tomography colonography (CTC) after incomplete optical colonoscopy.

INTRODUCTION: This study evaluated the role of computed tomography colonography (CTC) in patients who previously underwent incomplete optical colonoscopy (OC). We analyzed the impact of colonic lesions in intestinal segments not studied by OC and extracolonic findings in these patients. METHODS: Between January 2014 and May 2015, 61 patients with a history of abdominal pain and incomplete OC examination were studied by CTC. CTCs were performed by 320-row CT scan in both the supine and the prone position, without intravenous administration of contrast medium. In all patients both colonic findings and extracolonic findings were evaluated. RESULTS: Among the study group, 24 CTC examinations were negative for both colonic and extracolonic findings while 6 examinations revealed the presence of both colonic and extracolonic findings. In 24 patients CTC depicted colonic anomalies without extracolonic ones, while in 7 patients it showed extracolonic findings without colonic ones. DISCUSSION: CTC is a noninvasive imaging technique with the advantages of high diagnostic performance, rapid data acquisition, minimal patient discomfort, lack of need for sedation, and virtually no recovery time. CTC accurately allows the evaluation of the nonvisualized part of the colon after incomplete OC and has the distinct advantage to detect clinically important extracolonic findings in patients with incomplete OC potentially explaining the patient's symptoms and conditioning their therapeutic management. CONCLUSION: CTC accurately allows the assessment of both colonic and extracolonic pathologies representing a useful diagnostic tool in patients for whom complete OC is not achievable.

Terminal or truncal ligation of the inferior thyroid artery during thyroidectomy? A prospective randomized trial.

INTRODUCTION: Thyroidectomy is a common procedure in general and endocrine surgery. The technique of ligation of inferior thyroid artery (ITA) has been invoked as a possible cause of appearance of postoperative hypocalcemia. METHODS: We performed a prospective randomized study involving 184 patients undergoing total thyroidectomy to evaluate the differences of truncal ligation versus distal ligation of ITA in terms of postoperative hypocalcemia, vocal fold palsy, voice and swallowing impairment. The patients were divided into group A (trunk ligation of ITA) and group B (terminal branches ligation of ITA). RESULTS: We evaluated postoperative PTH and calcemia (immediate, 6 and 12 months after thyroidectomy), postoperative day of discontinuation of calcium and vitamin D supplementation, voice and swallowing complaints, evaluated by mean of two specific tests available in literature, day of hospital discharge. CONCLUSION: The only significant differences between the two groups were a higher immediate postoperative calcemia and a greater number of patients discharged without calcium and vitamin-D supplementation in the group B. In conclusion, no substantial differences were found between the two groups. The choice depends on the experience of the surgeon.

Partial Aneurysmectomy of Venous Aneurysms in Arteriovenous Dialysis Fistulas.

Upper extremity native arteriovenous fistulas (AVFs) continue to be the standard of care for hemodialysis patient's access. Although autogenous fistulas are superior to catheters and synthetic grafts, they are not without their own complications. One complication is venous aneurysms that can lead to skin erosion, bleeding, and site loss. Although traditionally repaired with ligation, interposition grafts, or stent placement, in this article, we discuss our experience with aneurysmorrhaphy utilizing a thoracoabdominal (TA) stapler. Thirteen aneurysms were treated with the TA stapler at a single, nonuniversity hospital for all patients from 2012 to 2014. The average aneurysm diameter was 3.6 cm, and the average fistula age was 57.9 months. There were no bleeding complications or recurrences. The primary patency was 80% at 6 months, with a primary assisted patency of 90% during the same time frame. Aneurysmorrhaphy with the TA stapler appears to be a safe and viable option for the treatment of venous aneurysms associated with AVFs.

Gastric polypoid lesions. Our experience.

BACKGROUND AND AIM: The Authors report the results of their experience on polypoids lesions of the stomach and on endoscopic polypectomies. PATIENTS AND METHODS: A study on 2000 OGD (oesophagogastroduodenoscopy) has been carried out on 95 patients with polypoid lesions. The authors have analysed the associations existing between histological type and symptomatology and localisation of the lesion and the status of the Helicobacter pylori and the risk of cancerization. The data were confronted with the ones already available. RESULTS: In the majority of the cases, the polypoid lesions were asymptomatic, the localization changed according to the histological type, with the antrum as the most affected area. The presence of Helicobacter pylori does not seem to be correlated to the lesion, except in the case of hyperplastic polyps. The percentage of risks of cancerization increased in case of adenomatous polyps. In one patient signet ring cell carcinoma within a gastric polyp was found. Gastric signet ring cell carcinomas are peculiar for their rarity as well as for the growth in polypoid lesions. CONCLUSION: We confirm the higher frequency of hyperplastic polyps and the correlation between histological type and localization. Endoscopic polipectomy is the first approach in gastric polyps, with lower risk of developing cancer. Only in selected cases, as in one in ours, it is advisable the surgery.

Numerical and experimental analysis of periodic patterns and sedimentation of lysozyme.

This paper deals with experimental investigation, mathematical modelling and numerical simulation of the crystallization processes induced by counter diffusion method of a precipitant agent in a lysozyme protein solution. Novel mathematical strategies are introduced to simulate the experiments and in particular to take into account the kinetics of the growth process and the motion of the crystals due to the combined effect of gravitational force and viscous drag if the sedimenting process is allowed (protein chamber free of gel). Comparison between experimental observations and numerical simulations in the presence of convection and sedimentation and without them provides a validation of the model. The crystal formation in gel results modulated in space. If the gel matrix is not present, convective cells arise in the protein chamber due to local inversions in the density distribution associated to nucleation phenomena. As time passes, these vortex cells migrate towards the top of the protein chamber exhibiting a different wave number according to the distance from the gel interface. The sedimentating particles produce a wake due to depletion of protein from the surrounding liquid. The models and the experiments may represent a useful methodology for the determination of the parameters and conditions that may lead to protein crystallization.

Effects of microgravity on the crystal quality of a collagen-like polypeptide.

(Pro-Pro-Gly)(10) is one of the most widely studied collagen polypeptide models. Microgravity crystal growth of (Pro-Pro-Gly)(10) was carried out in the Advanced Protein Crystallization Facility aboard the Space Shuttle Discovery during the STS-95 mission. Crystals were successfully grown in all experiments, using both dialysis and free-interface diffusion methods. The quality of the microgravity-grown crystals and of ground-grown counterparts was assessed by X-ray synchrotron diffraction. Microgravity-grown crystals exhibited a significant improvement in terms of dimensions and resolution limit. As previously reported, crystals were orthorhombic, space group P2(1)2(1)2(1). However, the diffraction pattern showed weak reflections, never previously measured, that were consistent with new unit-cell parameters a = 26.9, b = 26.4, c = 182.5 A. This allowed the derivation of a new model for the arrangement of the triple-helical molecules in the crystals.

Primary non-Hodgkin's lymphomas of the female breast.

The charts of 35 women with primary malignant non-Hodgkin's lymphomas (NHL) of the breast were retrieved from the files of the Istituto Nazionale Tumori, Milan, over a 30-year period (1957 to 1986). These cases represented 0.1% of the more than 25,000 primary malignant tumors of the breast treated during the same period. The median age of these patients was 57 years (range, 28 to 81 years). In most cases, the clinical diagnosis was carcinoma. The tumors were either Stage IE(48%) or IIE(52%) at presentation, and only two patients had B symptoms. The right breast was involved in 17 patients, the left breast in 14, and both breasts in two. According to the updated Kiel classification and the Working Formulation (WF) for Clinical Usage, three cases were lymphoplasmacytoid (immunocytoma) NHL (WF, A); three, centroblastic-centrocytic, follicular NHL (WF, B); four, centroblastic-centrocytic, diffuse NHL (WF, F); 17 centroblastic NHL (WF, G); three immunoblastic NHL (WF, H); two B-lymphoblastic NHL (WF, I); and one, a Burkitt-like NHL (WF, J). Treatment consisted either of a combination of surgery, radiation therapy, and chemotherapy or radiation therapy and chemotherapy. The follow-up period for 32 patients ranged from 6 to 161 months (mean, 45 months); 17 patients died of their disease. The prognosis appeared to be related to the histologic type and stage of the disease. Median survival periods were 63, 52, 42, and 47 months for centroblastic-centrocytic follicular, centroblastic-centrocytic diffuse, centroblastic, and immunoblastic NHL, respectively. The overall 5-year survival rate was 43%; the 5-year survival rate and the probability of freedom from progression at 5 years were, respectively, 61% and 50% for Stage I and 27% and 26% for Stage II disease.

Continuous intravenous infusion of TRH-T: clinical, cardiovascular and endocrinological effects.

Seven patients, six suffering from amyotrophic lateral sclerosis (ALS) and one from Friedreich ataxia, were treated with a placebo i.v. infusion during the first day and with TRH-T i.v. infusion at a rate of 2 mg/h for 8 h daily (total daily dosage 16 mg) on the 2 consecutive days. Continuous blood pressure (BP) and EKG monitorings were performed during 3 days infusion. Blood samples were collected for endocrinological evaluations. The neurological evaluation after acute TRH-T treatment showed an objective improvement in 3 of the 8. We found significantly higher values of systolic (max. difference of 10.1 mm Hg) and diastolic (max. difference of 8.8 mm Hg) BP than during placebo, beginning from the 5th h of the infusion (p less than 0.05). A trend in progressive increase of the heart rate (HR) reached statistical significance (p less than 0.01) at the 8th h of the second TRH-T infusion. The cardiovascular changes during the i.v. continuous TRH-T infusions were clinically irrelevant and never required the interruption of the treatment.

[Efficacy of flecainide in the treatment of ventricular tachycardia in the elderly. Dynamic electrocardiographic study]. / Efficacia della flecainide nel trattamento della tachicardia ventricolare dell'anziano. Studio elettrocardiografico dinamico.

The antiarrhythmic activity of flecainide has been tested in seven old patients (mean age 71 years) suffering from recurrent episodes of ventricular tachycardia. A basal 24-hour dynamic electrocardiogram was recorded for each patient and was repeated on day 5 of oral flecainide treatment (200 mg). A significant reduction (p less than 0.0001) of premature ventricular beats, of bigeminism (p = 0.0025) and of the episodes of ventricular tachycardia (p = 0.017) was obtained with plasma flecainide levels between 322 and 614 ng/ml. In addition, sinus rhythm was restored in two patients with stable atrial fibrillation and in the others ectopic atrial beats were markedly reduced. Among the ECG parameters examined, only QRS was significantly increased (p = 0.034). No significant change of blood pressure and laboratory parameters was observed. Untoward side effects were not observed.

[Annual variations in plasma testosterone levels in prepuberal subjects]. / Variazioni circannuali dei livelli di testosterone plasmatico in soggetti prepuberi.

40 prepubertal boys, aged 6 to 10 years, were studied from 1977 to 1979 in order to evidence any circannual rhythm in the testosterone plasmatic levels. The patients observed in the following life schedule: nocturnal rest from 2200 to 0600 circa, meals at 0800, 1300, 1900. Plasma samples were taken at 0800 and testosterone was assayed by RIA. Testosterone data were fitted a cosine function, by least square method in order to describe any rhythm and to estimate its parameters:mesor, amplitude, acrophase. A significative circannual rhythm in the testosterone levels is present (P= 0.008) with Acrophase in July (-198.38 degrees +/- 18.26 ES). Our study suggests that a significative testosterone rhythm, with circannual periodicity, is present also in prepubertal age.

[Effect of the degree of short stature on the type of response to tests of pituitary growth hormone reserve]. / Influenza del grado di deficit staturale sul tipo di risposta ai tests di riserva ipofisaria per l'ormone della crescita.

Two HGH (insulin and L-Dopa) stimulating tests were performed at time interval on 65 subjects with short stature (SDs between -2 and -4.5 according to Tanner) and on 18 control subjects, in order to study the influence of short stature degree on the HGH behaviour during the stimulating tests. The results obtained show that: 1. Statistically the short stature degree does not affect the response to HGH stimulating tests. 2. Two tests are necessary to avoid erroneous diagnosis of dwarfism caused by lack of HGH. 3. No response to HGH stimulating tests in subjects with normal stature may prove a discrepancy between the hormone measured radioimmunologically and its biological action.