Pilot study shows right ventricular diastolic function impairment in young children with obstructive respiratory disease.

AIM: This study determined cardiovascular impairment in young children with obstructive respiratory disease who were assessed using the opening interrupter technique (RINT). METHODS: This pilot study enrolled 41 children who had been referred to pulmonology and allergology specialists at the University of Catania, Italy, from March to July 2017: 23 (mean age 4.13 ± 0.62 years) had chronic coughs and wheezing and 18 controls (mean age 4.27 ± 0.66 years) had obstructive chest disease, but were otherwise healthy. Airway resistance was evaluated using RINT and cardiac function by studying the ejection fraction, pulmonary artery systolic pressure (PASP), tricuspid annular plane systolic excursion and tricuspid flow propagation velocity (TFPV). RESULTS: The RINT and PASP values were significantly higher in the patient group when compared to the controls, but the TFPV values were lower. A direct and significant Spearman's correlation coefficient (r) between RINT and PASP values was observed (r = 0.81). We found a significant inverse correlation between RINT and TFPV (r = -0.83), as well as TFPV and PASP (r = -0.78). CONCLUSION: This study showed that children with obstructive respiratory diseases had a major risk of cardiovascular impairment. Impaired diastolic function of the right ventricle occurred very early when airway resistance was abnormally increased.

Subclinic cardiac involvement in paediatric patients with celiac disease: a novel sign for a case finding approach.

The aim of the present work was to assess the prevalence of early cardiac involvement in children with celiac disease (CD), and the impact of a gluten free diet (GFD) on this issue. Sixty CD children was compared with a control group of 45 healthy children by an echocardiographic examination. CD patients were re-evaluated 1-year after 1-year GFD. Main outcome measures were ejection fraction (EF), fractional shortening (FS), left ventricular end-diastolic diameter (LVDD), left ventricular end-systolic diameter (LVSD), any regurgitating valve lesions. Mild cardiac involvement was found in 13 CD children and in one control (21.7% vs. 2.2%; p=0.003), and was secondary to regurgitation of mitral valve, aortic valve, pulmonary and tricuspid valve, or to impaired ejection fraction. CD children as compared to controls had significantly lower contractility indices, and higher left ventricular dimensions. In patients adhering to the GFD all valve regurgitations resolved, and the echocardiographic parameters significantly improved. Subclinical cardiac involvement in CD children is quite frequent, and GFD may exert a beneficial effect on the overall cardiac performance.

Troponin I as a biomarker of cardiac injury in neonates with idiopathic respiratory distress.

Troponin I (TnI), an inhibitory protein complex located on the actin filament of cardiac muscle, has become a specific marker of myocardial damage. Troponin has been studied in a wide range of clinical settings. However, many questions are still unanswered, especially in preterm neonates with the most common pathology at birth, such as idiopathic respiratory distress syndrome (IRDS). The aim of this study was to establish a reference range for cardiac TnI for healthy preterm infants and serum levels in sick preterm infants with IRDS. Echocardiography was performed and TnI serum levels were measured at a median age of 62 hours of life in three groups of healthy preterm infants (n = 10), and ventilated infants with moderate (n = 15) and severe IRDS (n = 15). Ventilated infants with idiopathic moderate IRDS had significantly different cardiac parameters (R/L ejection fraction, R/L stroke volume, R/L cardiac output; p < 0.05) and significantly higher cardiac TnI levels than healthy infants (0.037 versus 0.01 microg/mL; p < 0.05). Furthermore, infants with severe IRDS had higher TnI concentrations than infants with moderate IRDS (0.26 versus 0.037 microg/mL; p < 0.05). The results of this study show that increased TnI serum levels in sick preterm infants with IRDS are explained by myocardial injury or dysfunction due to impaired arterial oxygenation or reduction in cardiac output during treatment with mechanical ventilation. These results suggest that cardiac TnI may be a useful, specific marker for myocardial damage in preterm neonates with IRDS.

Endothelium and inflammation.

During the past decade, our understanding of the pathophysiology of coronary heart disease (CAD) has undergone a remarkable evolution. To date atherosclerosis is considered an inflammatory disease, whose the endothelial dysfunction represents an early key event. When the arterial endothelium encounters certain bacterial products or risk factors, such as dyslipidemia, vasoconstrictor hormones involved in hypertension, the products of glycoxidation associated with hyperglycemia, or proinflammatory cytokines derived from excess adipose tissue, these cells increase the expression of adhesion molecules that promote the sticking of blood leukocytes to the inner surface of the arterial wall. Once in the arterial intima these cells communicate with endothelium and smooth muscle cells, under the influence of mediators of inflammation and immunity, such as the cytokines and complements components, prostanoids and leukotrienes. Thus, the activated endothelium promotes the development of the atherosclerotic disease process, i.e., vascular inflammation and thrombosis by producing vasoconstrictor substances, by inducing the expression of adhesive receptors for leukocytes and platelets, the production of tissue factor and endothelin, and by increasing the production of the plasminogen activator inhibitor-1. Emerging data support the concept that assessment of endothelial vasomotion may be a useful biomarker for atherosclerotic vascular disease.

[Transient right ventricular hypertrophy in a newborn infant exposed in utero to nimesulide]. / Ipertrofia ventricolare destra transitoria in un neonato esposto in utero a nimesulide.

The use of non-steroidal anti-inflammatory drugs (NSAIDs) during pregnancy has been sometimes related to harmful cardiac effects secondary to the precocious closure in uterus of ductus arteriosus. This closure is linked to the inhibition of the cyclo-oxygenase (COX) enzyme that plays a key-role in the prostaglandin synthesis. Recently, it has been acquired that, among NSAIDs, nimesulide has a more selective action on the isoform COX-2 that is more strictly related to inflammatory phenomena. The isoform COX-1, instead, steadily expressed in tissues, would be responsible of continuous prostaglandin synthesis maintaining patent the ductus arteriosus in uterus. The case reported in this paper describes a remarkable right ventricle hypertrophy observed in a newborn whose mother, during the whole period of pregnancy, frequently used nimesulide as analgesic. Probably, in spite of its selective action on COX-2, the prolonged use of nimesulide has determined a closure of the ductus, inducing a functional fetal pulmonary hypertension that resolved after birth with the consequent regression of the right ventricular hypertrophy.

[Cerebral venous sinuses thrombosis in an infant with supramitral obstructive membrane associated with partial anomalous pulmonary venous return]. / Trombosi dei seni venosi cerebrali in un lattente con membrana sovramitralica ostruttiva associata a ritorno venoso polmonare anomalo parziale.

Cerebral venous thrombosis is quite rare in newborn infants, but probably its incidence is not well evaluated because clinical signs and cerebral echocardiography are not specific. We report the case of an newborn infant with massive cerebral venous thrombosis associated with heart malformation: supramitral ring and partial anomalous pulmonary venous connection in the superior vena cava.

Discharge criteria from intensive care unit in brain injured patients.

OBJECTIVE: We investigated the value of information on clinical features and intensity of treatment activity in the Intensive Care Unit (ICU) in predicting the need for further interventions after a patient is discharged from the Intensive Care Unit. Our aim was to assess if this could aid in making decisions about transfer to an Immediate Care Unit (sub-ICU). METHODS: We studied 39 patients with acute brain damage (traumatic or vascular causes). They ranged in age from 15 to 75 years and none had an associated spinal cord injury. The SAPS II, Glasgow Coma Scale (GCS), length of stay in ICU, duration of hospital stay and daily NEMS scores were recorded for each patient. We recorded the activities performed after transfer to an sICU, including complications that required active "life-saving" treatment. The role of each factor was assessed by using the odds ratio (OR), and with linear logistic regression. FINDINGS: 8 of the 39 patients developed a complication in the Sub-ICU. A linear logistic regression analysis demonstrated that the principal features having significant predictive value were:a) age, with an increase in risk of over 10 times for patients that were older than 50 (p=0.011);b) SAPS II scores > or = 50 points, with 24 times an increase in risk (p=0.002); and c) a GCS score < or =5 points, with an increase in risk of almost 7 times (p=0.024). INTERPRETATION: Complications in Sub-ICU are less likely in patients younger than 50 and who have SAPS II and GCS scores within predetermined limits. These indices can help in making decisions about discharge of a patient from ICU to Sub-ICU.

[Inflammatory tumors of the cecum in emergencies: surgical considerations and case report]. / I tumori infiammatori del cieco in urgenza: considerazioni di terapia chirurgica e caso clinico.

A cecal mass of uncertain etiology encountered during surgery for presumed appendicitis is a dilemma for the surgeon. The differential diagnosis of an unsuspected ileocecal mass must include neoplasm, diverticular disease, inflammatory bowel disease and severe appendicitis involving the ileocecal region. Right hemicolectomy is the gold standard for the treatment of the unsuspected ileocecal mass. The Authors report a case of inflammatory tumour of caecum secondary to appendicitis in a young adult.

[Cardiologic study of 10 patients with Duchenne muscular dystrophy(DMD): personal experience]. / Studio cardiologico in 10 pazienti affetti da Distrofia Muscolare Duchenne (DMD): esperienza personale.

Duchenne (DMD) and Becker (BMD) muscular dystrophies are an heterogeneous group of diseases characterized by a progressive muscular degeneration. The locus involved is localized on short arm of chromosome X (2.1) and the gene product has been called dystrophin. The total dystrophin deficiency leads to DMD while the reduction of dystrophin expression to BMD. The dystrophin has an elevated molecular weight (427 Kd) with different isoforms expressed in skeletal muscular system, central nervous system and myocardium tissue. The cardiovascular involvement is up to the muscular dystrophy: in the DMD patients it is secondary to the progressive course and it usually seen in wheel-chair patients. We have retrospectively evaluated through electrophysiology (EKG) and Echo (M-mode and 2 dimensional) studies 10 DMD patients to define the heart involvement.

[Cardiac involvement in Kawasaki disease. Our experience]. / II coinvolgimento cardiaco nella malattia di Kawasaki. Esperienza personale.

BACKGROUND: Kawasaki disease is an acute vasculitis of unknown cause that affects children under 5 years of age and in 20-25% of cases can cause coronary artery anomalies. Early therapy with intravenous gamma globulin and aspirin greatly decreases the risk of developing coronary anomalies, but sometimes coronary disease develops even in timely diagnosed and treated cases. METHODS: Personal experience in 41 children with Kawasaki disease is reported. Age was within three months and 5 years. All patients had electrocardiography and echocardiography at diagnosis, after 1 week, at the third, sixth, and eighth week, after six months and after 1 year from diagnosis. Echocardiographic cardiac anomalies were found only in 5 cases (12.19%). Average age in these cases was lower. RESULTS: In these cases coronary involvement developed in spite of early diagnosis and therapy (intravenous gamma globulin 400 mg/kg die and aspirin) electrocardiographic findings were not relevant or specific to coronary arteries anomaly. In three cases coronary anomalies regressed during the first year from diagnosis, the other two are still in the first year of follow-up. Nine children had a long term follow-up (6 to 9 years) and they were all normal except one with renal arteritis and another with slight mitral incompetence. CONCLUSIONS: The authors underline the role of clinical and instrumental follow-up that should be extended to all patients affected by Kawasaki disease with coronary anomalies even if early regressed.

[Cancer of the male breast]. / Il cancro della mammella maschile.

BACKGROUND: Male breast cancer is a rare type of neoplasm, being 1% of all breast tumors. An increasing relevance is given to genetic factors. Familiarity is present in 10% of cases reported in literature. Gynecomastia is frequently associated. Personal experience about this pathology is compared with the most recent data of the literature. METHODS: Nine cases of male breast cancer observed among 519 breast tumors operated between 1982 and 1997 are reported. Etiologic and prognostic aspects, surgical and complementary therapy of breast cancer in man are examined and the high rate of II-III stage patients, mostly ER+ and PR+ is underlined. Diagnosis is reached by ultrasonography and mammography, after an accurate clinical examination and confirmed by cytology after needle biopsy. The choice operation is total mastectomy with axillary lymphadenectomy according to Patey. RESULTS: According to our experience, 5 patients died for non neoplastic pathology, 1 patient lost at follow-up, 3 patients still alive after 66, 60 and 12 months respectively. CONCLUSIONS: Male breast cancer is similar to the female one, but characterized by a higher hormone receptors positivity. Our survival data have no statistic significance. It is still discussed if prognostic difference between men and women is present or not.

Vein of Galen aneurysmal malformation. Different clinical expressiveness. Three case reports.

The vein of Galen aneurysmal malformation (VGAM) is a rare cerebro-vascular disorder in neonates. It is characterized by an abnormal direct communication between one or several cerebral arteries and the vein of Galen. It may appear in the neonatal period or afterwards. Three cases of patients affected by VGAM with different clinical expression are presented. Two of them were treated successfully with endovascular embolization. It was not possible to provide the same treatment to the third patient for an intractable congestive heart failure already existing when VGAM was diagnosed.

[Post-influenza viral myositis: report of a case]. / Miositi virali post-influenzali: contributo casistico.

The inflammatory myopathies are a heterogeneous group of diseases due to autoimmunitary or infective causes. They are diagnosed by clinical electrophysiological features, and muscle biopsy. The autoimmunity group is subdivided in dermatomyositis, polimyositis and inclusion body myositis. The second group caused by viral genesis are called generally "myositis". The two groups have a different prognosis and therapy. The infective group denominated myositis have usually a viral etiology. We have evaluated 6 patients with viral myositis that are studied by physical examination, blood test (CK included), electromyographic pattern and muscle biopsy. The Authors emphasized the role of muscle biopsy for the diagnosis of this pathology and in particular for the myositis.

[A strangulated perineal hernia. A rare case of intestinal obstruction]. / Ernia perineale strozzata. Un raro caso di occlusione intestinale.

A rare case of intestinal obstruction due to strangulated perineal hernia, observed among 533 small bowel obstructions operated since January 1982 until December 1994 (0.2%) is described. Less than 100 cases are reported in literature. The etiologic, anatomical and clinical aspects of perineal hernia are examined. The reported case is of the primitive, congenital type caused by defect of the rectovaginal fascia fusion, in correspondence of the Douglas. Preoperative diagnosis is very difficult. Strangulation of this type of hernia is rare, the hernial sac surrounding tissue being elastic. In case of strangulation, symptoms of intestinal obstruction appear. In the reported case the preoperative diagnosis was "acute abdomen", because there was defence in hypogastrium and in the right iliac fossa. A straight abdominal radiography has not been performed. In the reported case the patient, a 22-year-old woman, was promptly operated and the incarcerated intestinal loop released. A regular postoperative period followed. The importance of a complete clinical examination, of a straight abdominal radiography and of a promptly performed operation is underlined.

[Cardiac involvement in tuberous sclerosis in the first months of life: physiopathologic and clinical aspects of cardiac rhabdomyoma]. / Il coinvolgimento cardiaco nella sclerosi tuberosa nei primi mesi di vita: aspetti fisiopatologici e clinici dei rabdomiomi cardiaci.

Cardiac rhabdomyomas are frequent in association with tuberous sclerosis and they are the first symptoms of Bourneville disease in fetal and neonatal period. Clinical findings of cardiac rhabdomiomas are quite heterogeneous: asymptomatic, cardiac murmur, cardiomegaly, heart failure or arrhythmias. Echocardiography can determine site, dimensions, numbers and haemodynamic consequences of cardiac tumours and their clinical evolution at follow-up. In this study the Authors report clinical findings in 9 cases of cardiac rhabdomyomas in newborn infants: 3 has familiarity for tuberous sclerosis and two of these had prenatal echocardiographic diagnosis; in the other 6 cases diagnosis was casual (1 in fetal period). All patients with prenatal diagnosis were asymptomatic. In other cases 1 had extrasystolic arrhythmias and five had only cardiac murmurs. Echocardiographic follow-up (6 months to 5 years) showed regression of number and dimension of cardiac masses in all cases. In 7 cases cardiac rhabdomiomas were associated with tuberous sclerosis.

[Intestinal obstruction due to Meckel's diverticulum. Description of three cases]. / Occlusione intestinale da diverticolo di Meckel. Descrizione di tre casi.

Among 588 small bowel mechanical obstructions operated since January 1982 until December 1996 at the Flajani Surgery Department and Emergency Department of the San Camillo Hospital in Rome, 3 male patients were operated for intestinal obstruction due to Meckel's diverticulum. In one case, obstruction was caused by a small bowel volvulus rolling on a Meckel's diverticulum, whose gangrenous extremity was "blocked" on caecum. In the other two cases, intestinal loops were incarcerated into an internal hernial ring constituted by the same diverticulum whose inflamed extremity was fixed to corresponding mesentery. We examined embryologic and clinical aspects of the pathology here considered, particularly its complications, obstruction being the most common in adult age. Diagnosis is often misunderstood, since a complicated Meckel's diverticulum simulates many other abdominal pathologies. A straight radiography and an ultrasonography of the abdomen may be useful to reach the correct diagnosis. We performed diverticulectomy, using a linear stapler and we underline the opportunity of this method. In young age laparoscopy resection is considered the gold treatment of this pathology by some authors. We didn't observe any mortality, although one of our patients was in a severe septic condition. It is necessary to examine the last ileal 100 centimetres when a suspected acute appendicitis is not initially found by operation. The opportunity of a promptly performed operation is underlined to prevent that such a benign pathology may induce also exitus.

[Physiopathologic findings and surgical treatment in transposition of great vessels: our experience]. / Rilievi fisiopatologici e trattamento chirurgico della transposizione dei grossi vasi: nostra esperienza.

Transposition of great vessels (TGV) is the most frequent neonatal cyanotic malformative cardiopathy. The Authors report their experience in surgical anatomical correction of transposition of the great arteries in 55 patients, 44 male and 11 female. Minimum age was 2 days, maximum 6 months. TGV was simple in 38 cases and associated with ventricular defect in 12 cases, with double outlet right ventricle in 3 cases, and with complex cardiopathy in 2 cases. There have been 11 deaths. The cause of death was: cardiac failure in 3 patients, myocardial infarct in 4 patients, respiratory insufficiency in 2 patients, and sepsis in 2 patients. There was one death by myocardial infarction and 1 asymptomatic ostial left coronary stenosis during follow-up (from 1 to 104 months). There were neither anastomotic pulmonary stenosis nor aortic valve incompetence. The authors review the literature on functional and anatomic correction of TGV and underline the importance of precocious anatomic together with early diagnosis, percutaneous atrioseptostomy and pharmacologic (PGE1) therapy in determining further reduction of mortality and to improve late outcome.

[Intestinal obstruction caused by paracecal hernia. 3 case reports and review of the literature]. / Occlusione intestinale da ernia paracecale. Descrizione di tre casi e revisione della letteratura.

The authors report three rare cases of intestinal obstruction due to paracecal hernia observed in 533 small bowel obstructions operated between January 1982 and December 1994 (0.6%). In our experience, all the cases occurred in old female patients. Less than 150 cases are reported in the literature. The authors examine paracecal hernia embryologic aspects to explain its pathogenesis: the rotation of primary intestinal loop determines final intestinal rapports. Preoperative diagnosis is very difficult. Transitory symptoms may appear months or years before intestinal obstruction; these occurrences suggest that internal hernias, like external ones, may spontaneously reduce. Straight abdominal radiographies, performed in all cases here described, demonstrate small bowel levels. There is disproportion between important subjective symptoms and objective finding of a large round bump localized in the right iliac fossa. Authors underline the opportunity of a promptly performed operation: in all our cases we released the incarcerated intestinal loops and sutured hernial foramen. One exitus occurred, on the 4th postoperative day, probably due to pulmonary embolism.

[Indications for percutaneous atrioseptostomy. Comparison of echocardiographic and fluoroscopic monitoring]. / Indicazioni alla atriosettostomia percutanea. Confronto fra monitorizzazione ecocardiografica e fluoroscopica.

Balloon atrial septostomy (BAS) is of established value in the management of several congenital heart diseases in the neonatal period. This procedure, which leads to creation of a tear in the membrane of the Foramen Ovalis using a balloon catheter, may be undertaken under fluoroscopic monitoring in catheter laboratory as well as under echographic control, even bedside. In this study we present our experience and discuss the indications to these two techniques. 91 neonates underwent to BAS; in 14 of them this was carried out under two-dimensional echocardiographic control in the intensive care unit. In all the patients BAS had good result, with clinical improvement in the majority of cases (97%) and a low rate (6%) of minor complications (such as transient supraventricular arrhythmias), without differences between fluoroscopic and ultrasound monitoring. The higher rate of mortality in the fluoroscopic monitoring group (2/91 = 2.2%) was thought to depend on an extremely critical presentation of the neonates. The echographic monitoring does not seem to offer any real advantage from the technical point of view, but the necessity of a prompt treatment of very ill patients emphasizes the advantages of a quickly and easily feasible procedure. Thus, we recommend the use two-dimensional echocardiographic imaging only in the very ill neonates in whom the septostomy can be more safely performed in intensive care unit bedside.

[Mechanical occlusion of the small intestines by gallstones. Our experience]. / Occlusione meccanica di tenue da calcoli biliari. Nostra esperienza.

The authors report their experience of biliary ileus, namely 8 cases out of 533 intestinal occlusions of the small bowel operated from 1982-1994. The patients included 5 women and 3 men with a F/M ratio = 1.7. Mean age was 79 years. Past medical history involving the biliary tract was only recorded in 3 cases. At the time of hospitalization occlusive symptoms had been present for between 2 and 7 days: pain in 7 patients, abdomen closed to gas and feces in 6, and vomiting in 7. Direct abdominal X-ray was only performed in 3 cases. A diagnosis of intestinal occlusion was made in 6 cases and biliary ileus in 2. During surgery it became clear that the occlusion was primarily localized at the level of the terminal ileum. Cholecysto-duodenal fistula was present in 5 cases. Enterolithotomy alone was performed in 5 cases. In 4 patients postoperative progress was normal leading to recovery. Two patients died: one immediately after surgery and the other on day 12. In conclusion, the authors confirm the increasingly frequent presence of cholecysto-duodenal fistula and the increasing age of patients, with a prevalence of females. They also emphasize the importance of an early diagnosis, supported by direct abdominal radiography so that surgery can be performed as rapidly as possible. Lastly, the need to perform cholecystectomy at a subsequent date is underlined.