Search

Left ventricular non-compaction (LVNC) is a myocardial disease characterized by a two-layered structure typically seen at the apical and lateral left portions of the ventricular myocardium, distal to the papillary muscles. While considered a rare disease, its prevalence in children is increasing due to the increased awareness of this condition and improved resolution of imaging techniques. The etiology is heterogeneous, ranging from inherited conditions to acquired diseases. Although many patients are asymptomatic, some patients may experience adverse events, including heart failure, arrhythmias, or thromboembolic events. Several echocardiographic or cardiac magnetic resonance imaging diagnostic criteria have been proposed for diagnosing LVNC. However, their application in children is significantly limited. This review aims to describe the clinical and genetic characteristics of children with LVNC and discuss the role of the proposed diagnostic criteria.

Pediatric cardiomyopathies are rare diseases, heterogeneous in clinical presentation, etiology and prognosis. Etiological diagnosis, where genetic analysis plays a key role, is of fundamental importance for defining diagnostic and therapeutic pathways. Furthermore, the identification of the genetic substrate represents a prerequisite for cascade screening in the proband's family members and to allow conscious reproductive choices. To date, genetic testing is performed with the analysis of gene panels (targeted panels) or with the study of the entire exome (whole exome sequencing) using next generation sequencing (NGS) technology. The great genetic heterogeneity and the temporal variability of the clinical manifestations lead to unique problems for pediatric cardiomyopathies, distinct from those of the adult, such as the possible indications for access to the test, the type of test to be used (exome or panel of genes), the importance of analyzing parents, especially in cases with neonatal onset; moreover, the correct execution of bioinformatics analysis and the interpretation of NGS data play a crucial role in the impact of the results on clinical management.

Mucopolysaccharidoses (MPS) represent a group of rare lysosomal storage disorders, with a heterogeneous clinical presentation in terms of inheritance (autosomal and X-linked recessive), age of onset (infants, children, and adults), systemic and cardiac manifestations (mild to severe disease forms). Evidence-based recommendations on the diagnosis and management of cardiovascular disease in MPS are scarce. GICEM (Gruppo Italiano Cardiologi Esperti Malattie Metaboliche) is a group of cardiologists, cardiac surgeons and pediatricians with a specific expertise in metabolic diseases including MPS. In this paper, we report our experience and recommendations on the diagnosis and management of cardiovascular aspects in MPS, with a tailored approach based on current evidence, and taking into account MPS phenotype (particularly, I, II, IVa, VI), age at presentation, and severity of systemic and cardiac manifestations.

Mild therapeutic hypothermia improves neurological outcomes after cardiac arrest by preserving brain function. It is currently under discussion the possibility that hypothermia may also provide a protective effect on cardiac function, in particular, by reducing the infarct size in patients with acute myocardial infarction complicated by cardiac arrest. Despite encouraging experimental and clinical data obtained so far may suggest a potential future indication in this population, routine use of therapeutic hypothermia in acute myocardial infarction patients needs further investigation and it is not currently recommended.

The biohazard represents a major occupational risk among workers in the health sector, this risk is not only exclusive for healthcare workers but involve also nursing students. The study reports data of a survey on injuries from accidental puncture in a group of 223 students of the third year of Nursing of Bari University. The 18% of students say they have suffered over the past 12 months an accidental puncture with sharp instruments. The cutting device most frequently involved is the needle from the syringe and insulin. The most at risk are the recovered and disposal of the needle. The biohazard in training is further compounded by factors such as lack of experience and skill manuals consolidated combined with a non perception of the risk. In the obligation of protection, training and information to students of Nursing, the University must implement programs aimed at both knowledge of the risks to which they are exposed, as well as security procedures to contain an emerging risk, which one of injury from sharp instruments, which are exposed young students not yet in employment.

Background: Medical progress in the world is divulged to peers and colleagues through books and scientific journals. It is thus linked not only to the quality of articles, but also to their type., Material and Methods: The type of articles published in three Italian periodicals (Annali Italiani di Chirurgia; Chirurgia; Minerva chirurgica) and three foreign periodicals published in English (Annals of Surgery; British Journal of Surgery; Surgery, Gynecology & Obstetrics) was compared. The articles were classified according to their potential contribution to medical progress, being divided into two main groups: research papers and simple case series or case reports. Other types of articles (reviews, description of surgical techniques, editorials etc.) were excluded from the study., Results: Both the total number and the percentage of research papers were markedly superior in the three foreign journals with respect to the three Italian periodicals. All the differences were highly significant., Conclusions: The number of research papers published in Italian periodicals should be increased. Greater attention should be paid to quality, at the expense of quantity, since serious research inevitably takes longer to perform.

There is international experience of Extracorporeal Membrane Oxygenation (ECMO) in children suffering acute Respiratory failure (eg. meconium aspiration syndrome); on the contrary there is a lack of consistent experience in pediatric ECMO application in cardiac surgery as bridge to cardiac transplantation and as heart mechanical support after postcardiotomy failure. In fact there are good standardization criteria for selection, inclusion and exclusion in ECMO application for neonatal respiratory failure; differently pediatric ECMO timing (that is when to start and to stop this ventricular support) is not clear and standardized. We present one case of pediatric ECMO application in a neonate, who underwent cardiac operation for pulmonary valve aplasia and who was impossible to wean from cardiopulmonary bypass.

From November 1968 to May 1990, 56 patients, 19 male and 37 female (mean age 49 +/- 14 years) underwent excision of an intracardiac myxoma (M). M was located into the left atrium in 48 (86%), the right atrium in 6 (11%), and the right ventricle in 2 (3.5%). Operation consisted of excision of the tumor together with its base of attachment. There were 2 early (3.5%) and 2 late (3.7%) deaths. Actuarial survival at 20 years is 91 +/- 4%. Non-invasive reevaluation has been carried out in 44 patients (84%), 39 with left atrial M, 4 with right atrial M, and 1 with right ventricular M. 2-D echocardiography ruled out tumor recurrence in all patients and showed, in those with left atrial M, a reduction in the size of the atrial chamber; on the contrary, left ventricular systolic diameter, diastolic diameter and ejection fraction were unmodified. In 7 patients (15.9%) a residual mild mitral insufficiency was disclosed. In 34 patients 24-hour electrocardiographic monitoring reevaluation showed a low incidence of major supraventricular arrhythmias, late postoperatively (short runs of paroxysmal supraventricular tachycardia in 3 patients, and atrial ectopic rhythm in 1). All these patients had undergone tumor excision by biatrial approach. Based on our results, we conclude that excision of intracardiac myxomas is curative and long-term survival is excellent. Non-invasive reevaluation of patients by 2-D echocardiography and 24-hour electrocardiographic monitoring is mandatory in order to promptly disclose possible complications, particularly tumor recurrence and arrhythmias.

The incidence of HBeAg in HBsAg positive hemodialysis patients in reported. 21.2% of hemodialysis patients have been resulted positive for serum HBsAg. 63.6% of HBsAg positive patients resulted to have HBeAg in their serum. Anti-HBe antibody was never found. The Authors emphasize the importance of HBeAg in hemodialysis patients to evaluate their infectivity.

The results are reported of an epidemiological assessment of 94 patients hospitalised with diagnosed acute viral hepatitis in the Infectious Disease Division of the S. Spirito Hospital, Casale Monferrato in the period march 1 1981, april 30 1982. Hepatitis was found to occur most frequently among young (under 30) males. There were more positive than negative HBsAg cases, most of the latter arising in heroin addicts.

Sera from seventy patients suffering for HBsAg positive hepatitis, twenty-five HBsAg blood-donors, and eighty-four healthy subjects with no history of hepatitis, negative for all markers of HBV infection, were tested for circulating IgM/HBsAg complexes by a radioimmunoassay. IgM/HBsAg complexes, present in acute hepatitis patients at admission, progressively decreased and could not be demonstrated in sera of the convalescent phase. No complexes were found in sera from subjects negative for HBV markers. The results obtained from sera from HBsAg positive blood donors are compared with HBeAg/anti-HBe system. The absence of IgM/HBsAg complexes seems to be a sign of good prognosis in HBV infection.

The prevalence of Hepatitis B Virus (HBV) markers was studied in 350 staff members from a Psychiatric Hospital, where a high prevalence of HBV markers was observed among patients. The prevalence of HBsAg, anti-HBs plus anti-HBc, anti-HBc alone and anti-HBs alone were determined to be: 2%, 15.1%, 4% and 0.3%, respectively. The prevalence of HBV markers was greater (although not significant) among women (24.7%) as compared to men (17.8%) (p greater than 0.05), and in the personnel with more than 20 years of employment (24.1%), as compared to those with less than 20 years (20% (p greater than 0.05). The highest prevalence of infection was observed in the department in which were admitted children and young adults. The overall prevalence of HBV markers was higher among staff members than in the blood donors of our area. Hospital personnel must know such epidemiologic conditions: active immunization with the Hepatitis B vaccine must be offered to hospital employees, firstly in those institutions in which a high prevalence of HBV infection has been documented.

Changes in susceptibility to four aminoglycosides (gentamycin, amikacin, tobramycin, sisomicin) of bacterial strains, isolated from in-patients with urinary tract infections at the "Santa Maria della Scala" Hospital of Siena, in the period January-April in three consecutive years (1980, 1981, 1982) were studied. The change susceptibility patterns were related to the use of the four antimicrobial drugs in the same periods. Emergence of antibiotic-resistant strains was seen after a wider use, whereas an increasing number of susceptible strains was observed after reducing use of antimicrobial drugs.

Between January 1980 and December 1983, 332 consecutive cases of acute hepatitis were observed in adult patients admitted to the Department of Infectious Disease and Gastroenterology of Siena. Sex and age of the patients, the presence of jaundice, the maximum value of the serum-glutamate-pyruvate-transaminase (SGPT) were considered. Serum specimens were tested for hepatitis B surface antigen (HBsAg), antibody against hepatitis B core antigen (anti-HBc) of the IgM class, antibody against hepatitis A virus (anti-HAV) of the IgM class, antibody against cytomegalovirus (CMV) of the IgM class, Paul Bunnel Davidshon reaction. Hepatitis A was diagnosed in 25 cases (7.5%). Hepatitis B in 167 (50.3%). Hepatitis due to CMV in 2 cases (0.6%). And, by exclusion, hepatitis non A, non B in 138 cases (41.6%). Male patients were affected with significantly higher frequency than female (p less than 0.01); the same was seen for young patients (14-30 years) compared to the older ones (31-50 years, and over 50 years) (p less than 0.01 in both). Biochemical investigation showed that hepatitis A and B had a significantly higher, maximum SGPT value than hepatitis non A non B (p less than 0.01 in both). Icteric patients were significantly more frequently observed among hepatitis A and B cases than hepatitis non A non B cases (p less than 0.01 in both).