"Not such a kid thing anymore": Young adults' perspectives on transfer from paediatric to adult cardiology care.

BACKGROUND: Transfer of adolescents with congenital heart disease from paediatric cardiology providers to specialized adult congenital heart disease (ACHD) care providers is becoming a standard practice. However, some paediatric cardiologists continue to provide care for their patients into adult life. Little is known about the perspectives of young adult patients who have been transferred to ACHD clinics versus those who continue to receive their cardiology care in paediatric settings. METHODS: Content and thematic analysis of structured telephone interviews with 21 young adults age 18-25 (13 transferred to ACHD clinic and 8 who had not transferred) was conducted to identify similarities and differences in patient characteristics of those in ACHD versus paediatric settings. RESULTS: There were no appreciable differences in gender, age, heart disease type, and independence between those transferred to ACHD care versus those not transferred. Participants in both groups were aware of differences between the paediatric and ACHD care settings and providers, with some favouring the familiarity offered by the paediatric setting and providers. Participants had varying views on parental involvement in their care; most of them had attended clinic appointments on their own. Those who had transferred to ACHD care acknowledged that it would take time to adjust to new relationships. Positive perspectives on actual or anticipated transfer to ACHD care included a growing sense of autonomy and responsibility, as well as access to reproductive information relevant to ACHD patients. CONCLUSIONS: The absence of patient characteristics distinguishing those in ACHD care versus those still followed in paediatric care suggests that system, provider, and parent factors, rather than patient factors, may account for patients' perspectives on transfer to ACHD care.

Atrioventricular septal defects with severe left ventricular hypoplasia. Staged palliation.

The natural and surgical history of patients with atrioventricular septal defect, severe left ventricular hypoplasia and right ventricular dominance is poor. We report a successful case of staged surgical palliation based on regulation of the pulmonary blood flow and creation of an unobstructed systemic outlet.

Cardiac rhabdomyoma: surgical treatment in the neonate.

Primary cardiac tumors are rare. Of the benign lesions, rhabdomyoma predominates in infants and children. The natural history of patients with cardiac rhabdomyoma is poor. About 50% die in the first month of life and 80% by 1 year of age. We report on the two youngest infants ever operated upon successfully for removal of symptomatic ventricular cardiac rhabdomyoma. The operations were performed through a left ventriculotomy in one and through a right ventriculotomy in the other during the first day of life.

Surgical bypass of the systemic atrioventricular valve in children by means of a valved conduit.

The surgical approach to relief of mitral stenosis in children is still a controversial problem. We describe our experience with four severely symptomatic children in whom a valved conduit from the left atrium to the left ventricle was successfully used to bypass a hypoplastic systemic atrioventricular valve. A left atrial-left ventricular extracardiac conduit was implanted in these patients with a hypoplastic mitral anulus and an adequate left ventricular chamber. There were no early or late deaths. Postoperative cardiac catheterization performed in all patients 1 month after the operation showed reduced size of the left atrium, a reduction of the left atrial-left ventricular gradient from a mean of 14 mm Hg to a mean of 5 mm Hg, and an increase of the left atrial outlet from a mean diameter of 10.7 mm to 28.7 mm (including the diameter of the native mitral valve plus the internal diameter of the valved conduit). The application of this unconventional operation in children with congenital or acquired stenosis of the systemic atrioventricular valve should be considered when the mitral valve obstruction cannot be relieved by conventional valve repair or replacement. Furthermore, the left atrial-left ventricular conduit does not preclude future alternative surgical options.

Total repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: an integrated approach.

OBJECTIVE: Predicting postrepair right ventricular/left ventricular pressure ratio has prognostic relevance for patients undergoing total repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. To this purpose, we currently rely on 2 novel parameters: (1) preoperative total neopulmonary arterial index and (2) mean pulmonary artery pressure changes during an intraoperative flow study. METHODS: Since January 1994, 15 consecutive patients (aged 64 +/- 54 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals were managed according to total neopulmonary arterial index. Seven patients with hypoplastic pulmonary arteries and a total neopulmonary arterial index less than 150 mm(2)/m(2) underwent palliative right ventricular outflow tract reconstruction followed by secondary 1-stage unifocalization and ventricular septal defect closure. The other 8 patients with a preoperative index of more than 150 mm(2)/m(2) underwent primary single-stage unifocalization and repair. The ventricular septal defect was closed in all cases (reopened in 1). In 9, such decision was based on an intraoperative flow study. RESULTS: Patients treated by right ventricular outflow tract reconstruction had a significant increase of pulmonary artery index (P=.006) within 22 +/- 6 months. Repair was successful in 14 cases (postrepair right ventricular/left ventricular pressure ratio = 0.47 +/- 0.1). One hospital death occurred as a result of pulmonary vascular obstructive disease, despite a reassuring intraoperative flow study. Accuracy of this test in predicting the postrepair mean pulmonary artery pressure was 89% (95% CI: 51%-99%). At follow-up (18 +/- 12 months), all patients are free of symptoms, requiring no medications. CONCLUSIONS: The integrated approach to total repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals by preoperative calculation of total neopulmonary arterial index, right ventricular outflow tract reconstruction (when required), and intraoperative flow study may lead to optimal intermediate results.

Ten-year follow-up after pediatric transplantation.

From November 1985 through December 1996, 128 patients aged 1 day to 18 years (mean age, 4.7 yrs; median, 3 years) were listed for heart transplant. Forty-seven (36.1%) died after a mean wait of 3.1 months, and 62 underwent transplant after a mean wait of 4 months. Two patients underwent retransplantation. The 1-, 5-, and 11-year actuarial survival rate for the patients who underwent heart transplantation is 68%, 62%, and 42%, respectively. The follow-up ranges from O to 132 months, with a mean follow-up of 39 months. At present, 36 patients, including the 2 who received a retransplant, are alive. Most of them have normal growth, development, and neurologic outcome.

Peripheral blood stem cells in children with solid tumors. Part I. Feasibility and application.

A method for collecting peripheral blood mononuclear cells following mobilizing chemotherapy in pediatric patients is described. The critical elements of the method included temporary heparinization of the patient to reduce citrate overload, and limiting extracorporeal circulation to 15% of the patient's blood volume using packed red blood cells and albumin. A median of 0.9 x 10(8) mononuclear cells/kg per collection were harvested in 40 collections from eight patients with only one episode of fever and chills. Peripheral blood stem cells were reinfused into six of these patients with refractory/recurrent pediatric tumors after intensive chemotherapy. Bone marrow reconstitution followed with a mean of 30 days (19-38) for absolute neutrophils and 48 days (32-275+) for platelets. Previous chemotherapy did not appear to affect peripheral blood stem cell efficacy in reconstituting chemotherapy-ablated bone marrow.

IL-8, IL-6 and ICAM-1 in serum of paediatric patients undergoing cardiopulmonary bypass with and without cardiocirculatory arrest.

BACKGROUND: The aim of the present study was to evaluate the systemic inflammatory response to CPB in paediatric patients undergoing surgical correction of congenital heart diseases. EXPERIMENTAL DESIGN: comparative investigation. SETTING: paediatric cardiology hospital INTERVENTION: ICAM-1, IL-8, and IL-6 production were analysed before and during CPB, and after surgery in 9 paediatric patients, submitted to cardiocirculatory arrest (Group A); and in 11 without cardiocirculatory arrest (Group B). MEASURES: ICAM-1, IL-8, and IL-6 production were analysed from arterial samples before and during CPB, and after surgery. RESULTS: In group A vs group B a significant increase of IL-8 was detected during (297+/-250 vs 11+/-19 pg x ml(-1), p<0.001) and after (100+/-230 vs n.d. pg x ml(-1)) surgery and was correlated with the duration of operation (r=0.759; p=0.0001) and clamping time (r=0.738; p<0.05). After surgery in group A, IL-6 levels (35+/-43 pg x ml) were higher than those in group B (2+/-5 pg x ml), and a good correlation was observed between IL-6 and duration of aortic clamping (r=0.714; p=0.048), cardiac arrest, (r=0.714; p=0.048), and length of surgery (r=0.867; p=0.04). CONCLUSIONS: In children who underwent CPB with cardiocirculatory arrest cytokine production seems related to duration of operation and amplified by ischemia-reperfusion phenomena.

[Use of mesoglycan in venous pathology]. / Uso del mesoglicano in patologia venosa.

Twenty-five female patients suffering from primary venous insufficiency of the lower limbs underwent parenteral and oral treatment with mesoglycan for 3 months. In addition to an evaluation of the subjective and objective parameters linked to venous insufficiency, all patients underwent lower limb venous echo colour-Doppler and videocapillaroscopy using an optic probe in a perimalleolar or periulcerous site. At the end of treatment, all patients reported an improvement in subjective parameters, which was confirmed by a reduction of distal edema in 22 out of 25 cases. There was also an improvement in capillaroscopic findings (reduction of edema of pericapillary connective tissue, reduction of capillary and venular ectasia.

[Reliability of N.M.R. employed in evaluating refrigerating mixtures organic solvents R 22 (Author's transl)]. / Validità dell'impiego della risonanza magnetica nucleare nello studio dei miscugli frigoriferi solvente organico-R 22

In a previous work the existence of an hydrogen bond between the difluoromonochloromethane (R 22) and the diethylesters of alpha-omega dicarboxylic acids was pointed out. In the present paper, the influence on absorption of substituents for the hydrogen of the CH2 has been studied in a single compound (diethylmalonate). The results show that electron acceptor substituents obstruct the forming of the hydrogen bond while electron donor substituents improve it.

[Telemetric surveys in plant pathology and for taking the census of ungulates]. / Il telerilevamento in patologia vegetale e nel censimento degli ungulati.

Two new applications of the remote sensing techniques in the territory management which make use of a thermal scanner, are described here: the first concerned with the vegetal pathology field and the second with the big game censusing the Castelporziano farm. The former constitutes an improvement of the usual techniques either for its quick performance or for being an uncurrent intervention on plants, the latter allows to realize censuses also in thickly bushy or woody lands, where the usual methods of counting "at sight", besides to need a large staff are not much reliable.

[Celiac disease: correlation of different diagnostic methods (author's transl)]. / La malattia celiaca: valutazione comparativa delle varie metodiche diagnostiche.

The results of four diagnostic tests have been critically evaluated (xylosemia, serum Ig A level, steatorrhea, X-Ray of the digestic tract) in 80 cases of atrophy of the small intestinal mucosa discovered by biopsy, and in 20 cases in which the biopsy has proved normal. In the celiac subjects the best correlation with the biopsy was found with xylosemia (90%), followed by serum Ig A (87%), by X-Ray of the digestive tract (86%) and finally by steatorrhea (59%). Even in cases without histological alteration of the mucosa, and xylosemia proved abnormal in 70% of the cases, the seric IgA resulted higher greater than 2 delta in 35%, the steatorrhea in 29% while the X-Ray of the digestive tract has never given a false diagnosis. The presence of false negative or positive results in all four tests (except the radiological tests) reconfirms the absolute necessity for biopsy of the digestive tract in the diagnosis of celiac disorder, and has led the AA. Carry out whenever possible the xylosemia, the steatorrhea and the serum IgA level because in none of the 80 cases of celiac disorder has there been a false negative result in all three such tests at the same time.

Active retinitis in an infant with postnatally acquired cytomegalovirus infection.

Congenital cytomegalovirus (CMV) is frequently associated with active retinitis. In contrast, in the immunocompetent neonate with postnatally acquired CMV infection retinitis is rarely present and usually does not progress. We describe the case of an infant with postnatal CMV infection and active retinitis diagnosed at 20 days of life. Owing to the rapid progression of the retinitis, therapy with intravenous ganciclovir was performed, with prompt regression of the retinitis. Therapy was then continued with oral valganciclovir for one further week. Although very unusual, CMV retinitis has to be taken into consideration in neonates with early postnatally acquired CMV infection, as an early diagnosis and treatment may be crucial to avoid visual impairment.

Thermodynamic study on the effects of beta-cyclodextrin inclusion with anilinonaphthalenesulfonates.

Thermodynamic parameters and stoichiometries for the binding of anilinonaphthalenesulfonates to beta-cyclodextrin are obtained from steady-state fluorescence intensity and anisotropy measurements. Specifically, formation constant, enthalpy, and entropy values are obtained for complexes of beta-cyclodextrin with eight different substrate molecules at five different temperatures and six different pH values, and their associated errors are given. We propose an explanation of the relative magnitudes of the values obtained with regard to the geometry of the substrate and the importance of the various noncovalent interactions responsible for the complexation.