beta-Galactosidase from Aspergillus niger in adult lactose malabsorption: a double-blind crossover study.

An assessment was made of the efficacy of a beta-galactosidase, obtained from Aspergillus niger and added to intact milk, in decreasing lactose malabsorption and intolerance. Sixteen adult patients with malabsorption and intolerance to this sugar were studied in a double-blind crossover study vs. placebo. A 5-hour hydrogen breath test was used to assess malabsorption of lactose contained in 400 ml milk. When compared with placebo, the addition of exogenous lactase to intact milk caused a statistically significant reduction in the maximum breath H2 concentration (P less than 0.01) and in the cumulative H2 excretion (P less than 0.005). In the same way, the cumulative index for gastrointestinal intolerance was significantly lower (P less than 0.005) after the ingestion of lactase-added milk. This study demonstrates that enzyme replacement therapy, with beta-galactosidases obtained from Aspergillus niger, is effective in decreasing lactose malabsorption and its consequent intolerance in adult subjects with lactase deficiency.

Incidence of residual defects determining the clinical outcome after correction of tetralogy of Fallot: postoperative late follow-up.

The postoperative results in 150 patients who had repair of tetralogy of Fallot (mean follow-up, 10.2 +/- 2.6 years) were defined as good in 71.3%, fair in 20.7%, and unsatisfactory in 8.0% on the basis of clinical criteria. Ninety-six percent of the patients are socially active, 92.0% have a good exercise tolerance, and 79.3% participate in sports. Data from 78 postoperative hemodynamic studies show mild right ventricular dysfunction in almost all patients examined. Peak systolic right ventricular/left ventricular pressure ratio decreased in the postoperative period in 65.4% of patients, remained unchanged in 3.8%, and increased in 30.8%. Minimal residual anomalies can modify the prognosis substantially. Of our patients, 53.3% of those with a shunt (p less than 0.05), 37.5% of those with stenosis (not significant), and 53.8% of those with pulmonary incompetences (p = not significant) have fair or unsatisfactory results. The associated defects are particularly unfavorable. Rhythm and conduction disturbances have resulted in fair or unsatisfactory results in 65.1% of patients. Such a disturbance occurring soon after bypass must be considered an incremental risk factor: 52.9% of patients seen with rhythm and conduction disturbances show the same disturbances later (p less than 0.001).

Mastectomy and immediate breast reconstruction: oncological considerations and evaluation of two different methods relating to 88 cases.

The authors consider 88 cases of mastectomy and immediate breast reconstruction mainly performed using the skin expander plus prosthesis method or latissimus dorsi myocutaneous flaps. At the same time, 53 patients underwent contralateral mastopexy for symmetry. The mean follow-up was 21 months (range 2-102). Progressive disease was observed in nine cases: one patient presented scar relapse, one axillary relapse, two contralateral tumor, two contralateral tumor and distant metastasis, three distant metastases and one death from distant metastasis. Reconstruction complications were capsular contracture in 12 cases, infection in nine, skin necrosis in two, skin expander breakage in three and implant dislocation in one. The final result was judged good in 54 cases, fair in nine, poor in 11 and unevaluable in 14. In conclusion immediate breast reconstruction does not seem to interfere with the disease or oncological therapy. After analysing separately, and comparing the results and complications of the two main techniques used, latissimus dorsi seems to be the most reliable method in the majority of cases but skin expanders can be a good technique in patients with small and firm breasts and without complicating risk factors.

Vascular rings and slings. Diagnosis and surgical treatment of 49 patients.

Forty-nine infants with symptomatic vascular rings and slings, ranging in age from 20 days to 12 months, required surgical intervention between 1973 and 1984. The following anomalies were present in our patients: double aortic arch with left descending aorta (14), double aortic arch with right descending aorta (6), anomalous innominate artery (13), right aortic arch with aberrant left subclavian artery (4), left aortic arch with aberrant right subclavian artery (10), aberrant left pulmonary artery (pulmonary sling) (2). All the babies had symptoms related to compression of the trachea and/or esophagus. Four patients required temporary tracheostomy in the early postoperative period; 1 patient, affected by a pulmonary sling, required tracheal resection and anastomosis, for severe tracheomalacia. There was one hospital death in a patient with severe tracheal compression from an anomalous innominate artery and brain damage as a result of metabolic problems. Forty-eight patients survived and follow-up ranged from 3 months to 11 years. For each type of vascular anomaly encountered, and based on personal experience, we have outlined a diagnostic scheme allowing an accurate morphological definition and a subsequent surgical procedure.

Goretex patch aortoplasty for coarctation in children: nuclear magnetic resonance assessment at 7 years.

From 1979 to 1990, 56 children ranging between 4 days and 16 years of age (mean 73 +/- 51 months) underwent Goretex patch aortoplasty for coarctation of the aorta. The mean weight at operation was 20.2 +/- 3.5 kg (range 3.3-42 kg). Forty-two patients had primary repair, and the remaining 14 had reoperation for recoarctation. The aorta was opened through a standard left thoracotomy, the posterior fibrous ridge was partially excised when it was prominent, and a large patch from a Goretex tube was sutured into place. The postoperative complications were as follows: paradoxical hypertension in 14 cases, massive haemorrhage due to aortic wall rupture in a diabetic child, and intestinal bleeding in 1 case. There were no early deaths and only 1 case of late death, which was not related to coarctation repair (mortality rate 1.8%). The average follow-up was 48 +/- 26 months. Continuous wave-Doppler examination at rest showed no arm-leg systolic gradient in 52 cases and a gradient of about 15 mmHg in 4 cases. Graded exercise testing showed only 1 case with an arm-leg gradient higher than 35 mmHg. Nuclear magnetic resonance (NMR) imaging, performed on 26 patients at a mean of 7 years from operation, showed excellent morphology and size of the aortoplasty. No cases of recoarctation or late aneurysm formation were found. We conclude that Goretex patch aortoplasty can be performed effectively and safely in children. Nuclear magnetic resonance provides high resolution imaging of the coarctation repair site.

Clinical evaluation of oxidative stress and myocardial reperfusion injury in pediatric cardiac surgery.

BACKGROUND: To evaluate oxidative stress and myocardial damage after aortic crossclamping release (ACCR) during cardio pulmonary bypass (CPB) in children two parameters were investigated: total glutathione (GSH) and its oxidoreductive reactions (GSH/GSSG) as expression of oxidative stress, and plasmatic turnover of myocardial taurine (TAU) as expression of cell damage. METHODS: The study was divided in two periods: 1) first period: analysis of oxidative stress and myocardial damage in 18 children. 2) Second period: evaluation of myocardial cell protection by controlled anterograde low oxygen warm reperfusion (ALOWR) before declamping. Twenty-one children were divided in two groups: not receiving (Group 1, 9 patients) and receiving (Group 2, 12 patients) ALOWR. RESULTS: In the first period GSH values increased significantly after onset of mechanical ventilation (MV) in vein, after CPB start in artery and after ACCR in coronary sinus. Moreover TAU turnover in aortic and coronary sinus blood increased significantly after ACCR. In the second period, Group 2 showed a lower oxidative stress after ACCR, while no differences were observed in TAU turnover. CONCLUSIONS: 1) Assessment of TAU and GSH levels can be considered a good method to clinically evaluate myocardial injury during cardiac surgery. 2) MV and CPB can induce oxidative stress before aortic clamping and can decrease the physiologic scavengers. Therefore, to prevent that depletion, the strategy of these techniques must be adapted to the patient and to his cardiac disease. 3) Intramyocardial TAU turnover is not significantly modified by the reperfusion technique. 4) ALOWR can reduce myocardial oxidative stress and can improve heart recovery after the cardioplegic arrest.

Quantitative and qualitative evaluation of blood salvaged after extracorporeal circulation (ECC) in paediatric heart surgery. Study of biochemical, morphological and structural variations of RBC after ECC and after salvaging of ECC circuit priming blood.

The salvaging of ECC circuit priming blood is essential for reducing the morbidity related to homologous blood transfusions and the importance of this technique is inversely proportionate to the age and weight of the child. In infants, the washing and centrifugation of blood not only drastically reduce the risk of contracting blood-transmitted diseases and cut management costs, but are also of considerable hemodynamic importance, producing a rapid normalization of the patient's hematocrit and hemoglobin and balancing the O2 consumption/demand ratio. The marketing of miniaturized salvagin devices with 55 ml bowls by Dideco has made possible the recovery of small quantities of blood, so as to normalise the hematic crisis and permit the application of total hemodilution in low-weight patients. The salvaged blood shows an average hematocrit of 52.7+/-9.7% (max 68.1%) and an average hemoglobin of 17.6 +/- 2.9 g/dl (max 20.7 g/dl), and maintains its structural components, osmotic resistance, concentration of intraerythrocytic hemoglobin and mean corpuscular hemoglobin all intact. Washing with isoosmotic and isoionic hydroelectrolytic solutions normalizes the ionic situation in the post-operative period and activated blood salvaging after Extracorporeal Circulation. The use of solutions without nutritional substances results however in a considerable fall in the number of enzymes in the intraerythrocytic metabolic glucide chain (G6PDH: -40.7 +/- 14.3% p<0.001), (PK: -23.8 +/- 20.5% p<0.03). This drop may be responsible for erythrocytic morphological alterations (echinocytic change) and probably for the release of hemoglobin from the red blood cells. Washing with isoionic, isoosmotic solutions containing G5% and adenine could, at least in theory, improve the quality of the salvaged blood, by normalizing the morphology and the volume of the RBC and by increasing the hematocrit.

[Prevention of hemorrhagic complications following cardiopulmonary bypass]. / Prevenzione delle complicazioni emorragiche conseguenti a bypass cardiopolmonare

Isolated primary fibrinolysis following cardiopulmonary bypass was fully prevented by using a modern washing technique for the reusable metal parts of the extracorporeal circuit: immersion in 20% caustic soda for 4 hr and in a detergent solution for a further 4 hr prior to rinsing and sterilisation. Reduction of bleeding from 558 to 465 cc/m2 was attributable an improved perfusion technique, early diagnosis and radical cleaning of the circuit. The disappearance of fibrinolysis, however, was entirely brought about by more thorough cleansing of the reusable metal parts.

[Circulation support in left thoracotomy for descending thoracic aorta diseases in pediatric patients]. / L'assistenza circolatoria in toracotomia sinistra nella patologia dell'aorta toracica discendente nel paziente pediatrico.

BACKGROUND: Complications of surgery of the descending thoracic aorta could include neurological problems such as paraparesis and paraplegia. Intestinal ischemia and acute renal failure could occur, too. During the cross-clamping time, the perfusion of the distal aorta by means of extracorporeal circulatory assistance can be useful to protect the spinal cord and the splanchnic organs and to reduce the incidence of these untoward events. The aim of this study was to verify the efficacy and suitability of this type of circulatory assistance in a pediatric age group. METHODS: From January 1996 to March 2000, in the Pediatric Cardiac Surgery Department of the Gaslini Institute (Genoa, Italy), 6 patients (mean age 39 months, mean weight 14.4 kg) were treated using extracorporeal circulatory assistance through the left thoracotomy. Indications for surgery included the removal of a migrated device previously inserted in order to close a patent ductus arteriosus in 1 patient, recurrent coarctation in 2 patients, and native coarctation with a poor collateral circulation in 3 patients. RESULTS: The early and late mortality rates as well as the morbidity rates (both neurological and splanchnic) were nil. CONCLUSIONS: Extracorporeal circulatory assistance can be helpful in pediatric patients and it can prevent neurological and renal injuries in cases of a) coarctation of the aorta with a poor collateral circulation and low distal pressure (< 40 mmHg during aortic occlusion); b) recoarctation with complex anatomy; c) other pathologies requiring prolonged descending thoracic aorta cross-clamping.

[Occurrence of hospital infections in a department of pediatric heart surgery]. / Studio di incidenza delle infezioni ospedaliere in un reparto di cardiochirurgia pediatrica.

The incidence of nosocomial infections (NI) and the related risk factors in a Department of Pediatric Cardiovascular Surgery were studied, during a 6 months period. 155 successive admissions were considered. Nosocomial infections were 17 (11%), nosocomial colonizations 18 (11.6%). The most important risk factors for nosocomial infections were: age, cyanosis, duration of hospitalization, hospitalization in Intensive Care Unit and central venous catheter only as a risk factor for sepsis. The most important risk factors for nosocomial colonizations were: tracheal intubation and central venous catheter. In 4 cases the NI was related to nosocomial colonization (2 sepsis, 1 pneumonia, 1 wound infection). The most frequently isolated microorganisms were Pseudomonas aeruginosa and Staphylococcus spp. The Authors found that a longer than 5 days period of antibiotic prophylaxis did not reduce the incidence of nosocomial infections.

[Role of extracorporeal circulation in reversible acute cardiorespiratory insufficiency in neonatal and pediatric intensive care]. / Ruolo della circolazione extracorporea nell'insufficienza cardiorespiratoria acuta reversibile in terapia intensiva neonatale e pediatrica.

In the present study we try to analyze indications, contra-indication, side effects and limits of the use of extracorporeal circulation in neonatal and pediatric patients affected by reversible acute cardiorespiratory failure. The greatest experience on this technique has been achieved in the neonatal age (about 6000 newborns have been treated until now), while the employment in the pediatric age appears more recent (about 500 patients). Moreover, we focused on the drop-in criteria for neonatal and pediatric patients and on the clinical aspects and laboratory findings which can anticipate the surviving rate; the latter appears in any case much greater in the neonatal patient compared to the other pediatric ages.

Characterization of Aspergillus oryzae fermentation extract effects on the rumen fungus Neocallimastix frontalis, EB 188. Part 2. Carbon source utilization and effects on zoospore production.

The effect of a commercial Aspergillus oryzae fermentation extract on the utilization of carbon source and zoospore production by the rumen fungus Neocallimastix frontalis EB 188 was determined. In addition, the composition of a soluble extract prepared from the commercial product was analyzed. This extract was added to N. frontalis EB 188 cultures grown on a variety of substrates and periodically assayed for protein, enzymes, zoospore production, and carbon source utilization. The powdered product contained 93% dry matter, more than 3,000 A. oryzaespores per gram, and did not contain strong buffers or high concentrations of salt. Measurable concentrations of DNA, protein, carbohydrate and several enzymes including cellulase and amylase were also found. Soluble extract increased fungal physiology and treated cultures produced significantly higher levels of supernatant protein and enzymes including amylase, cellulase and beta-glucosidase. The fungal response depended on culture carbon source. However, culture zoospore production was increased regardless of substrate provided. Culture utilization of glucose was more rapid in treated cultures, yet high levels of the extract greatly inhibited glucose utilization.

Surgical management of complete atrioventricular canal associated with tetralogy of Fallot.

Between 1984 and 1993, 12 children with an atrioventricular canal and tetralogy of Fallot underwent surgical repair. The mean(s.d.) age at operation was 58(18) months, and the mean(s.d.) body weight 15(4) kg. Nine patients underwent 11 palliative procedures. The ventricular septal defect was closed through a combined (right atrial and right ventricular) approach in nine cases, and through a right atrial approach in three, using a prosthetic patch with a wide anterior extension, secured with a running suture. The 'ostium primum' defect was closed with a separate prosthetic patch in 11 cases (double-patch technique). Right ventricular outflow obstruction was relieved by a composite infundibular patch (seven cases) or a transanular patch (five). There were four hospital deaths (33%). These were caused by low cardiac output in three cases and infection in one (three deaths occurred in patients with a transanular patch). One patient has so far died during follow-up. Assessment at 50(36) months by echo-Doppler showed moderate-to-severe 'mitral' regurgitation in three cases, and moderate 'tricuspid' regurgitation with right ventricular dysfunction in one case. Two patients have required further surgery.

Characterization of Aspergillus oryzae fermentation extract effects on the rumen fungus Neocallimastix frontalis, EB 188. Part 1. Zoospore development and physiology.

Experiments were performed to determine the effect of Aspergillus oryzae (AO) fermentation extract on zoospore development in the rumen fungus Neocallimastix frontalis EB 188. Powdered product, or liquid extract prepared from such powder, was added at the recommended value for supplementation in dairy cattle. Stationary and stirred cultures were periodically sampled and assayed for extracellular and intracellular protein and enzymes, gas production, zoospore production and maturation, and carbon source utilization. Soluble extract increased fungal physiology when grown in stirred vessels or stationary cultures. Treated cultures produced higher levels of enzymes (nearly double). Mobile zoospores matured into germination entities more rapidly in treated cultures, and when powdered product was used, nearly 3 times more motile zoospores were produced at 56 h of fungal growth. Levels of the intracellular enzyme malate dehydrogenase increased by 6-fold in the presence of powdered product. Product wheat bran carrier used as soluble extract or powder had very little effect on fungal cultures. Medium cellulose was completely hydrolyzed in all cultures but this occurred earlier in those containing AO treatment.

Activation of the intracellular glutathione system by oxydative stress during cardiopulmonary bypass and myocardial perfusion.

The glutathione (GSH) system is the main defence of tissues against free radicals and red blood cells (RBC) are the most efficient sites for GSH redox cycle activation. Total GSH was assayed during cardiopulmonary bypass (CPB) in RBC and serum from the coronary sinus, peripheral arteries and veins in 18 children corrected of their cardiac defect. Our conclusions are: (1) RBC-GSH redox cycle is activated during heart ischaemia and reperfusion; (2) the activation of intracellular GSH system is preponderant compared with the extracellular one; (3) variations in intraerythrocytic total GSH during heart ischaemia and perfusion are detectable in peripheral veins and arteries, which can be the convenient sites for monitoring changes in the GSH cycle; and (4) increased total GSH levels are present in RBC before aortic crossclamping: at the beginning of mechanical ventilation in veins and, when CPB is started, also in arteries.