Search

The objective of this study was to evaluate the cost-effectiveness of voriconazole versus amphotericin B as primary therapy for immunocompromised patients with aspergillosis. We used a pre-existent model in which two cohorts of patients received voriconazole or amphotericin B. The patients were followed for 12 weeks. The analysis was conducted from the perspective of the Italian National Health Service. We considered life years gained (LYG) and direct medical costs. LYG were based on the results of an international clinical trial and direct medical costs were based on Italian treatment patterns. Benefits were discounted at 3%. Sensitivity analysis on key clinical and economic parameters were performed. LYG with voriconazole compared with amphotericin B were respectively 13,87 and 11,36 years. The expected cost was € 25.366 with voriconazole and € 26.218 with amphotericin B. This cost-effectiveness analysis suggests that voriconazole is a dominant strategy compared with amphotericin B for the primary treatment of aspergillosis in immunocompromised patients.

Objective: Our study was designed to evaluate the effects of 2 dosage schedules of recombinant interferon (IFN)-α (IFNα-2a and IFNα-2b) in reducing serum ALT and eradicating serum hepatitis C virus (HCV) RNA in β-thalassaemic patients with chronic hepatitis C. Design: 38 Sicilian β-thalassaemic patients (22 males and 16 females) received intramuscular IFNα-2a (Roferon-A®; Roche) 5 MU/m2 3 times weekly for 6 months, followed by 3 MU/m2 3 times weekly for a further 6 months. 13 Sardinian β-thalassaemic patients (7 males and 6 females) received intramuscular IFNα-2b (Intron®; Schering-Plough) 3 MU/m2 3 times weekly for 12 months. Parallel control groups (n = 20 and n = 8, respectively) did not receive IFNα. All patients received continuous subcutaneous desferoxamine infusion. Results: 24 (63%) Sicilian patients had a positive clinical response to IFNα-2a therapy. Two different patterns of response were apparent: (i) early and progressive decrease in ALT values until stable normalisation; and (ii) slower reduction of ALT values, which fluctuated on the way to normalisation. Five (21%) patients relapsed during the 12-month follow-up period. ALT levels decreased early in 5 (38%) Sardinian patients and one patient (20%) relapsed during the 12-month follow-up period. In the control groups, ALT values spontaneously normalised in 3 (10%) untreated patients. None of the patients treated with IFNα developed anti-IFNα antibodies. Viral clearance was demonstrated in 19 (50%) of 38 patients in the Sicilian group and 4 of 13 patients (31%) in the Sardinian group. Conclusion: Treatment with intramuscular recombinant IFNα-2a 5 MU/m2 3 times weekly for 6 months, followed by 3 MU/m2 3 times weekly for 6 months, appeared to be more effective than intramuscular IFNα-2b 3 MU/m2 3 times weekly for 12 months.

Thirty-eight clinical strains of Helicobacter pylori were isolated from patients with chronic gastritis and gastroduodenal ulceration, and their susceptibility to macrolide antibiotics (roxithromycin, flurithromycin, azithromycin, erythromycin) in combination with proton-pump inhibitors (lansoprazole and omeprazole) and bismuth subcitrate was assayed. Chequerboard titration was used to analyse the results of antimicrobial interactions and showed that the activity of macrolides was enhanced by combining them with lansoprazole, omeprazole or, to a lesser extent, bismuth subcitrate. While the interactions between erythromycin and the proton-pump inhibitors or bismuth subcitrate were always additive, the combinations of roxithromycin-lansoprazole, flurithromycin-omeprazole and azithromycin-lansoprazole acted synergically on 82%, 60% and 60% of H. pylori strains, respectively. These results may, in part, account for the enhanced clinical efficacy of macrolides administered with proton-pump inhibitors in the treatment of H. pylori-associated diseases.

Thromboembolic (TE) events have been frequently reported in β-thalassemic patients in association with known risk factors such as diabetes, complex cardiopulmonary abnormalities, hypothyroidism, liver function anomalies, and postsplenectomy thrombocytosis. In a recent survey involving 9 Italian thalassemic centers, we identified 32 patients with TE episodes in a total of 735 subjects, of whom 683 had thalassemia major and 52 thalassemia intermedia, corresponding to 3.95 and 9.61%, respectively. There was a great variation in localization: the main one (16/32) was CNS, with a clinical picture of headache, seizures and hemiparesis. Other localizations were the pulmonary (3 patients), mesenteric (1 patient) and portal (2 patients) sites. There were 6 cases of deep venous thrombosis (2 in the upper limbs, 4 in the lower ones). Intracardiac thrombosis was found in 2 subjects and clinical and laboratory signs of DIC were observed in 2 others during pregnancy. Since our patients with TE events present a statistically significantly higher incidence of associated dysfunction (cardiomyopathy, diabetes, liver function anomalies, hypothyroidism) than those without TE events (50 vs. 13.8%), we suggest close monitoring of those patients who are at higher risk of developing TE events because of the presence of one or more of these predisposing factors.

Concurrently in the West of Venezuela, they have come proposing technicians to improve the recovery of oil weighed through of new processes of thermal stimulation that do not use water steam as agent of heating. In this work it is considered the injection of hot gasoil the reservoir and exists the thermal stimulation process's physical mathematical foundation, based on the several mechanisms of heat transference as convection and conduction, as a whole with the mechanism of transference of matter for liquid diffusion – liquid, which one, it based in: A Balance of calorific energy, through it is determinated distribution of temperature in the reservoir, and the heat losses to the adjacent formations evaluated by the model of Marx and Langengein, besides the model of production defined by Boberg and Lantz. A Balance of matter ruled by Fick's Law of the diffusion oil-solvent, and the equation of difusitivity that governs the transitory behavior of pressure defined by Everdingen and Hurst. The stability of the simultaneous solution of the differential equations system is acceptable, due the founded solutions are analytical type. Operationally the efficiency of the stimulation and the field practice, they will be key points that there will allow the successful execution of the planned process, through the wells completation with sand control filters, sensors of pressure and temperature, besides the thermal equipments of completation that requiere the thermal processes. To illustrate the field application of the proposed technology, there was selected the reservoir Bachaquero-01. The results obtained in several running of sensibilities, indicate that it is achieved an increase obtains in the mobility factor of the oil at reservoir conditions of 10,5 times superior than the original one. Giving cycles of production of approximately 10 months, for a stimulated average rate with hot solvent of 850 BN/day versus stimulated average day with steam of 380 BN/day, in an arrangement of conformated wells by two horizontal ones in parallel. The obtained results indicate that the heat solvent injecting technology to the formation is more favorable alternative than the steam alternate injection process, due it is predicted a major optimized recovery of oil by cycle.

One of old and most important West Venezuelan Oil fields is the LL-03 reservoir which has accumulated more than 1200 MMBN and actually represent the 30% medium oil daily production of Exploitation Unit "La Salina", Maracaibo District, PDVSA. This reservoir is characterized for contain 30% of shale with not consolidated sand at low pressure with 450 psi at 3500 feet of depth with interleayer of clay and high permeability with lost of severe circulation risks, in order to develop the reserves of 24° API oil it was designed and execute the secondary recovery project called Fase IV through instrumented horizontal wells into low-pressure zone. Due the depleted conditions of this reservoir it was begun the search of new alternatives for perforation fluids competitive at technical and economic levels, being one of the main challenges to ensure the success in the construction and completion for extend horizontal wells. After obtained the formulation of the optimal fluid to use it was come to design the directional plan and the mechanical completion for sand control, being considered in addition the use of permanent downhole gauge for pressure and temperature readings, conforming therefore the instrumented well. The stability of the system, the effectiveness of the saline seal and operational practice allowed the successful perforation of 3 instrumented horizontal wells (2 injectors and 1 producer) with the formulated saline fluid in a horizontal section of 2699 feet average without observe hole instability problems having completed the wells with mesh screen for sand control and permanent downhole gauge for pressure and temperature readings in a concentric string, after removing the filter cake generated by the perforating fluid it was obtained 22 months of continuous and stable production of 900 BNPD results Vs 450 BPPD expected for PB-763 well, opening opportunities for the perforation of 3 wells in similar conditions and it was begun the project for perforation of the first multilateral well in this same reservoir.

Osteoporosis in beta-thalassemia major has emerged as a topic of interest since optimized transfusion regimens have increased life expectancy and quality in these patients. Although the pathogenesis of thalassemic osteopathy is multifactorial, the evidence of an increased resorption phase suggests that the use of antiresorptive drugs such as bisphosphonates can be considered a valuable therapeutic strategy to reduce bone turnover and the risk of fragility fractures. We compared the effects of long-term cyclical clodronate therapy (300 mg intravenous infusion every 3 weeks for 2 years) and of an active placebo (calcium 1 vitamin D) on bone mass and bone turnover in 30 male patients with beta-thalassemia major. We also tested the possibility of using quantitative ultrasound (QUS) for assessing bone involvement in thalassemic osteopenia and in monitoring the response to antiresorptive therapy. Broadband ultrasound attenuation (BUA) was significantly reduced in patients with beta-thalassemia major as compared to healthy controls. In calcium and vitamin D-treated patients, a significant decline in spine, femoral, and total body areal bone density was observed. In the patients given intravenous clodronate we measured a substantial stability of bone mass, which was not significantly changed at the end of the study. The urinary excretion of deoxypyridinoline (a marker of bone resorption) showed a progressive significant decline throughout the study period in clodronate-treated patients. No significant change was observed in BUA values in both groups of patients. These results indicate that intermittent intravenous clodronate administration was not able to increase areal bone density in our thalassemic patients. Moreover, this is the first study to have assessed the usefulness of broadband ultrasound measurements in beta-thalassemia major.

This paper describes 12 history cases of the successful applications of Alternate Path (shunt tube) Technology in wells in Bachaquero Lago, Tía Juana Lago and Lagomedio fields, in Lake Maracaibo.The use of divergent tubes combined with packoff in these wells, allowed to complete two zones in a single trip (stimulation or packing in the superior zone and packing in the inferior zone), reducing the number of pipe trips in each well and therefore, reducing the operational risks and drilling costs. This type of completion will also allow independent isolation in anyone of the zones in the event of watering problems, allowing selective production.In all the cases, successful gravel packing were achieved as well as 45% saving in drilling time, compared with conventional completion. In all the cases productivity was higher than the initial estimate, without formation sand production, confirming the success of the application.This technology provides a reliable and profitable alternative for the completion of two or three simultaneous zones.

The effects of an alternative chelation program in thalassemic patients with severe iron overload are investigated. The schedule of treatment, feasible at home, consists in the administration of deferoxamine intravenously (100 mg/kg/die 8 hours 10 days a month), followed by 50 mg/kg/die subcutaneously in the remaining 20 days of the month. The results in 34 patients who underwent this program over 8 months are reported. After intensive chelation therapy serum ferritin and transaminase levels were significantly lower, and daily urinary iron excretion values were significantly higher when compared to the levels observed before the treatment. After the period of study, echocardiography revealed an ejection fraction (EF) significantly higher in 15 out of 34 cardiopathic patients. In conclusion, the alternative chelation program is effective in reducing iron overload of thalassemic patients, protecting them also against cardiac disease.

The pathological conditions characterized by microcytosis are reviewed and their pathophysiological mechanisms also at the molecular level are described. Moreover clinical, haematological and laboratory findings for differential diagnosis are discussed. Finally, the most efficacious schedules for the treatment of iron deficiency anaemia are reported.

The case of a young man effected by homozygous beta-thalassemia is reported who had serologic findings of a prior HBV infection and who presented with clinical and biochemical acute HBV infection probably caused by HBV reactivation after allogeneic bone marrow transplantation. The patient's clinical history suggests that HBV can persist without serological findings of HBsAg and HBV-DNA in persons previously infected by HBV and that HBV reactivation can occur 2 years after allogeneic bone marrow transplantation, as a result of immunosuppressive therapy or an HCV activation.

We evaluated the immune status with respect to HBV and the immune response to readministration of HBV vaccine in a series of 20 patients with homozygous beta-thalassemia, aged 6-23 years (mean age: 13.0 +/- 4.2) who had undergone allogeneic bone marrow transplantation (BMT). Thirteen of them (group A), had received three doses of plasma-derived HBV vaccine from 7 to 5 years before BMT and 4-5 weeks after the last dose of vaccine, they had had high serum levels of HBV antibodies (anti-HBs). The remaining seven patients (group B) had had clinical symptoms and laboratory evidence of HBV infection in childhood with markedly elevated serum of anti-HBs. Before revaccination, a significantly lower percentage of patients (P < 0.005) with seropositive levels of anti-HBs was observed in group A than in group B. After administration of the second dose of HBV vaccine the percentage of subjects with protective levels of anti-HBs rose to 100% in both groups of patients even if the geometric mean of titers of anti-HBs increased more significantly in group B patients than in group A. We conclude that the serum levels of anti-HBs afforded by HBV vaccine administered from 7 to 5 years previously are very low and probably non-protective in most beta-thalassemic patients after allogeneic BMT, and that at least two doses of HBV vaccine should be readministered from 18 to 24 months after BMT to achieve adequate and long-term protection from HBV.

Sixteen patients with thalassaemia major were treated with subcutaneous desferrioxamine (DF) 50 mg/kg/d, 5 consecutive days a week, for 8 weeks. Every other week the total dose was administered by 12 h infusion pump or by rapid injection of the same dose (25 x 2 mg/kg) twice a day. The two methods of DF administration produced no significant differences in urinary iron excretion. No significant changes in serum ferritin levels were observed at the end of the study. Compared with continuous infusion, rapid injection is equally efficacious, does not induce serious side-effects, is better accepted by the patients, and can improve their compliance to the iron-chelating therapy.

A 10-mo-old baby girl with homozygous beta0 thalassemia and alphaJOxford, presenting the clinical picture of homozygous beta thalassemia is described. Hemoglobin electrophoresis showed three bands: the first two with the mobilities of hemoglobin Hb A2 (1%) and Hb F (69%), respectively, the third migrating a little faster than Hb A (30%). About 30% of her alpha chains were J Oxford which, bound to her gamma chains, produced a new alkali-resistant hemoglobin, alpha2 J Oxford gamma F2, which has not been described previously. Hemoglobin synthesis in vitro showed the absence of beta chain synthesis and an alpha/non-alpha ratio of 2. The patient's father was heterozygous for both the Hb J Oxford and beta0 thalassemia genes, the mother a carrier of beta0 thalassemia; four other relatives were carriers of Hb J Oxford, and one was a carrier of beta thalassemia.

The degree of imbalance in beta(0)-Th and beta(+)-Th as well as the frequency of the two forms in Sicilian beta-thalassemic subjects have been studied. The hemoglobin synthesis in Rietti-Greppi-Micheli disease (RGMD) and in the beta-thalassemia trait has also been studied. In an unselected thalassemic population, about 30% have been found to be beta(0)-Th. Both groups of beta(0)-Th and beta(+)-Th showed severe imbalance with alpha/non-alpha ratio of 4.22 +/- 1.88 (SD) and 3.46 +/- 1.36, respectively. This difference was not statistically significant. In RGMD the alpha/non-alpha ratio was 2.12 +/- 0.36 while in the beta thalassemia trait it was 1.76 +/- 0.35.

Haemoglobin synthesis was studied in bone marrow erythroblasts and in reticulocytes of 4 children with β°- thalassaemia major and of 7 children with β+ -thalassaemi

In patients with betao thalassaemia from Ferrara, beta globin mRNA sequences are either absent or structurally abnormal while in betao thalassaemia in Catania, beta globin mRNA sequences are present. In deltabeta thalassaemia there is a deletion of beta-like globin genes, while in betao Catania DNA, no beta globin gene deletion is detectable.

In the south-east of Sicily 23 children from 14 unrelated families have been diagnosed as suffering from haemoglobin Lepore. Such a high incidence shows that Sicily is an important focus of haemoglobin Lepore. The results of haematological and biosynthetic studies in 18 carriers of Hb Lepore and in five double heterozygotes for Hb Lepore and beta-thalassaemia are presented. In the carriers the haematological and biosynthetic data are compared with carriers of beta-thalassaemia, while the five double heterozygotes are compared with beta o- and beta +-thalassaemia major subjects. In the carriers of Hb Lepore no synthesis of delta beta-chains was observed in peripheral blood cells; in fact we found a peak in the bone marrow. Double heterozygotes with circulating nucleated red cells showed delta beta-chain synthesis in peripheral blood.

A 3-year-old child of Sicilian origin was found to have a severe form of Cooley's anemia. Investigations were extended to other members of her family. In three, a rare β-chain structural Hb variant, Hb G San Jose [β7 (A4) Glu→Gly], was observed: in the father of the porposita heterozygosity for the abnormal Hb was found to be coexistent with β° thalassemia; two sisters had lowered MCV and MCH values and levels of the abnormal Hb significantly lower than in other heterozygotes for Hb G San Jose. The α-chain/total β-chain synthesis ratios suggest an α-thalassemic-like effect. Their mother had lowered MCV and MCH values, an Hb A2 level in the upper limit of the normal range, and a balanced α-chain/β-chain synthesis ratio. Therefore, the possibility of coexistence of an α thalassemia trait with a β thalassemia trait in the mother of the proposita and with Hb G San Jose heterozygosity in the two sisters who had lowered levels of abnormal Hb is discussed.

Eight Sicilian patients with Hb H disease and their families have been studied. The standard haematological tests and the alpha/beta chain synthesis ratios showed significantly different results in the patients with Hb H disease as compared with alpha thalassaemia carriers, except for Hb A2 values. There was no significant difference in the mean RBC, MCV, Hb A2, Hb A1 and Hb F of alpha thalassaemia carriers compared with normal controls. On the contrary significant difference was found between the mean alpha/beta chain synthesis ratio of alpha thalassaemia carriers and that of the normal controls; however, the extensive overlapping of alpha/beta values between these two conditions make this parameter insufficiently discriminant. No correlation was found between MCV, MCH, RBC and alpha/beta chain synthesis ratio in patients with alpha thalassaemia trait, suggesting that the ratio cannot be used to distinguish between carriers of a mild gene ('silent' carrier) and carriers of the more severe alpha thalassaemia gene. A possible genetic model for alpha thalassaemia in Sicily is presented.

Hb G San Jose (beta7 glu leads to gly) was studied with respect to oxygen affinity, Bohr effect, surface activity in dilute aqueous solutions, mechanical precipitability, heat stability and its ability to copolymerize in the deoxy form with Hb S. Oxygen affinity, Bohr effect, and polymerization with Hb S were found to be identical to those of Hb A when studied under the same conditions. However, surface activity and mechanical precipitation rates of the oxyconformers closely resembled those of oxyhemoglobin S. Hb G San Jose was also found to be slightly more unstable with heat than Hb A, although the instability was not detected by the usual incubation method of 1 hr at 50 degrees and higher temperatures were needed to elicit this difference. It is concluded that the ability to polymerize and the presence of increased surface activity are distinct and separable attributes of hemoglobin mutants. The finding that mixtures of Hb S and Hb G San Jose gel like mixtures of Hb S and Hb A supports the conclusion that only one beta 6 Val combining site per tetramer is required for polymer formation.

Lymphocyte subpopulations were studied in 20 hypertransfused patients with beta-thalassaemia major, some of whom had been splenectomised. B-lymphocytes were normal but T-lymphocytes were decreased in all patients. The T-cell count was lower in the splenectomised patients than in the nonsplenectomised ones. In the former, the active rosette-forming lymphocytes were also diminished, but the difference was not significant. In all patients the percentage of null cells was greater and the activity of K-cells increased compared with controls.

In a prospective study, 144 patients undergoing thoracotomy were randomized to two groups: In 71 cases cryoanalgesia was applied intraoperatively to the intercostal nerves above and below the incision to relieve postoperative pain, and 73 (control group) received bupivacaine-adrenaline intercostal blockade at the end of the operation. The amount of administered narcotic and mild analgesics, the visual analogue pain scores, the need for further intercostal blockade and the number of postoperative bronchoscopies to clear retained secretion were significantly less in the cryoanalgesia group than in the controls. There were no late nerve complications after cryoanalgesia, which is recommended for routine use in thoracotomy.

The degree of imbalance in beta(0)-Th and beta(+)-Th as well as the frequency of the two forms in Sicilian beta-thalassemic subjects have been studied. The hemoglobin synthesis in Rietti-Greppi-Micheli disease (RGMD) and in the beta-thalassemia trait has also been studied. In an unselected thalassemic population, about 30% have been found to be beta(0)-Th. Both groups of beta(0)-Th and beta(+)-Th showed severe imbalance with alpha/non-alpha ratio of 4.22 +/- 1.88 (SD) and 3.46 +/- 1.36, respectively. This difference was not statistically significant. In RGMD the alpha/non-alpha ratio was 2.12 +/- 0.36 while in the beta thalassemia trait it was 1.76 +/- 0.35.

The precocious fusion of the proximal humeral epiphysis has been reported in adolescents with thalassemia major. We evaluated the incidence of this skeletal abnormality in two groups of patients characterized by a different timing of the hypertransfusional regimen (i.e., before or after 10 years of age). Precocious epiphyseal fusion was significantly more common among patients who started hypertransfusional regimen after 10 years of age. Starting hypertransfusional regimen since early life may prevent epiphyseal fusion as a result of a permanent block of marrow proliferation.

A 3-year-old child of Sicilian origin was found to have a severe form of Cooley's anemia. Investigations were extended to other members of her family. In three, a rare beta-chain structural Hb variant, Hb G San José [beta 7 (A4) Glu to Gly], was observed: in the father of the porposita heterozygosity for the abnormal Hb was found to be coexistent with beta o thalassemia; two sisters had lowered MCV and MCH values and levels of the abnormal Hb significantly lower than in other heterozygotes for Hb G San José. The alpha-chain/total beta-chain synthesis ratios suggest an alpha-thalassemic-like effect. Their mother had lowered MCV and MCH values, an Hb A2 level in the upper limit of the normal range, and a balanced alpha-chain/beta-chain synthesis ratio. Therefore, the possibility of coexistence of an alpha thalassemia trait with a beta thalassemia trait in the mother of the proposita and with Hb G San José heterozygosity in the two sisters who had lowered levels of abnormal Hb is discussed.

A 10-mo-old baby girl with homozygous beta0 thalassemia and alphaJOxford, presenting the clinical picture of homozygous beta thalassemia is described. Hemoglobin electrophoresis showed three bands: the first two with the mobilities of hemoglobin Hb A2 (1%) and Hb F (69%), respectively, the third migrating a little faster than Hb A (30%). About 30% of her alpha chains were J Oxford which, bound to her gamma chains, produced a new alkali-resistant hemoglobin, alpha2 J Oxford gamma F2, which has not been described previously. Hemoglobin synthesis in vitro showed the absence of beta chain synthesis and an alpha/non-alpha ratio of 2. The patient's father was heterozygous for both the Hb J Oxford and beta0 thalassemia genes, the mother a carrier of beta0 thalassemia; four other relatives were carriers of Hb J Oxford, and one was a carrier of beta thalassemia.

The characteristics of the fetal haemoglobin (HbF) in two children with osteopetrosis and high levels of HbF have been studied. The structural analysis of the gamma chains demonstrated a fetal Ggamma/Agamma ratio. HbF was distributed inside only 30% of the peripheral red blood cells. In vitro globin chain synthesis studies showed that there was balanced globin chain production, despite the increased level of HbF.

A Sicilian family is described in which the alpha-thalassaemia gene is interacting in several members with beta-thalassaemia resulting in a balanced alpha/beta chain production ratio. In one patient, affected by homozygous beta-thalassaemia, the presence of alpha-thalassaemia resulted in a less severe clinical expression of the disease, less marked imbalance in the alpha/non-alpha ratio, and a lower level of HbF. Further studies of haemoglobin synthesis are needed to clarify the complex genetic picture that results from the interaction of different forms of thalassaemia.

Haemoglobin synthesis was studied in bone marrow erythroblasts and in reticulocytes of 4 children with beta O-thalassaemia major and of 7 children with beta +-thalassaemia major. In patients with beta O-thalassaemia the gamma/a ratio was found to be lower in bone marrow than in peripheral blood. On the contrary, in patients with beta + thalassaemia the beta + gamma/a ratio was more balanced in bone marrow, where the beta-chain synthesis was higher, than in reticulocytes. This last result could be explained by the presence of an abnormal m-RNA for beta-chains in beta +-thalassaemia.

Nine Sicilian children known to be deficient in glucose-6-phosphate dehyrdrogenase (G6PD) were studied to see if there were anomalies of bactericidal activity in peripheral blood phagocytes. The type of deficiency was established. The G6PD levels in the leukocyte were found to be 26% of the controls (0.094 +/- 0.03, normal controls 0.360 +/- 0.12). The Michaelis constant for NADP and glucose-6-phosphate (G6P) was lower than the control. Conversely, the utilization of the analogous 2-deoxyglucose-6-phosphate (2dG6P) and galactose-6-phosphate (Ga16P) was higher. The thermostability of the enzyme in the deficient subjects was lower and the pH optima (8 and 9.5) were different from the controls. An identical electrophoretic pattern was found in both normal and deficient subjects. The bactericidal activity in the deficient subjects was normal. There was no difference in the results of nitroblue tetrazolium (NBT) tests in either group.

In patients with betao thalassaemia from Ferrara, beta globin mRNA sequences are either absent or structurally abnormal while in betao thalassaemia in Catania, beta globin mRNA sequences are present. In deltabeta thalassaemia there is a deletion of beta-like globin genes, while in betao Catania DNA, no beta globin gene deletion is detectable.

The presence of cholelithiasis was diagnosed by ultrasonography in 10 patients with thalassemia major aged 16 to 33 years. Other 10 patients aged 7 to 19 years showed acalculous cholecystopathy. Serum liver enzymes and ferritin levels, as well as splenectomy do not influence significantly the production of gallstones. Significant differences were observed in the age of patients with gallstones when compared to subjects without gallstones or with acalculous cholecystopathy. Although, in the last years, the high transfusional regimen has decreased the incidence of cholelithiasis, the frequent liver disease could be a cause of acalculous cholecystopathy in younger thalassemic patients.

A case of double heterozygosity for A gamma delta beta-thalassaemia and A gamma G gamma delta beta-thalassaemia was found during a screening programme in Sicily. The proband, a 4-year-old girl, showed a clinical picture of thalassaemia intermedia. Hb F (85.12% by the Singer method) was G gamma A gamma type. The parents and the brother were delta beta-thalassaemia carriers. Structural analysis of Hb F showed both G gamma and A gamma chains in the father, but only A gamma chains in the mother.

The results of hematological and biosynthetic studies in 15 subjects carriers of Hb Lepore and in 5 double heterozygotes for Hb Lepore and thalassemia are presented. In the carriers the hematological and biosynthetic data are compared with carriers of thal., while the five double heterozygous patients were compared with ° and + thal. major subjects. In the carriers of Hb Lepore no synthesis of chains was observed in the peripheral blood cells, on the contrary we found a peak in the bone marrow. Double heterozygous subject with circulating nucleated red cells showed in the peripheral blood chains synthesis.