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D Baronciani

Publications and source records attributed to D Baronciani.

At least 37 records · Page 2Linked to original sources
PubMed1997

Screening for developmental dysplasia of the hip: from theory to practice. Collaborative Group DDH Project.

OBJECTIVE: To evaluate an organizational model for neonatal population screening for developmental dysplasia of the hip. METHODS: In 4648 neonates born in six hospitals of the Lombardy region, screening for developmental dysplasia of the hip was done using the Ortolani-Barlow maneuver and ultrasonography. RESULTS: The frequency of positive results of clinical and ultrasound examinations carried out in the hospitals varied considerably as a result of difficulties in the Ortolani-Barlow test reproducibility and in the low sensitivity of the clinical examination when compared to ultrasonography. Neonatal screening results implied a large number of subjects with a IIa hip, according to Graf's system; as these subjects require follow-up, the cost of this type of screening is high. Ultrasound findings were normal at 69 days of life in 88% and 75% of subjects, respectively, with unilateral and bilateral type IIa hip. CONCLUSION: This study evaluated various organizational models for screening (for different time periods and for selected populations) in relation to the cost-benefit ratio and demonstrated the different problems that still impede identification of a correct screening model.

Hip Dislocation, Congenital↗
PubMed1996

Marrow transplantation for patients with thalassemia: results in class 3 patients.

Thalassemia patients can be categorized as class 1 (minimal liver damage and iron overload), class 3 (extensive liver damage from iron overload), and class 2 (intermediate). These categories are prognostic for treatment outcome after marrow transplantation. Class 3 patients have more transplant-related mortality than other patients. This study examines transplantation outcome for class 3 patients. Records were reviewed of 215 patients in class 3 who received transplants in Pesaro from HLA-identical related donors between May 1, 1984 and May 1, 1994. The influence of pretransplant, peritransplant, and posttransplant variables on survival, relapse, and transplant-related mortality was examined by product-limit and proportional-hazards multivariate analysis. Age and conditioning regimen were influential on survival, and regimens with less than 200 mg/kg cyclosporine (CY) were associated with 5-year survival probabilities of .74 and .63 patients younger than 17 years and older patients, respectively. Transfusion history and regimen were influential on rejection with 5 year probabilities of .53 and .24 in patients who received less than or greater than 100 red blood cell transfusions before transplantation and regimens containing less than 200 mg/kg CY. Results of transplantation for patients with advanced thalassemia treatment have improved with the introduction of conditioning regimens with less CY. This has been associated with an increase in rejection (particularly in patients who have received < 100 red blood cell transfusions before transplant). Efforts at reducing the rejection rate by modifying the conditioning regimen should be concentrated on younger patients who have received a small number of transfusions. Patients with thalassemia who have HLA-identical family members should be transplanted before they are in class 3.

Adolescent↗
PubMed1996

Bone marrow transplantation in a thalassemia patient with congenital heart disease.

We report a thalassemia patient suffering from congenital transposition of the great arteries, surgically corrected according to Mustard technique at the age of 4 months, who underwent bone marrow transplantation. Despite a syncopal episode occurring during the first day after marrow infusion the transplant was successful. Thirty-two months later, normalization of hematologic parameters was observed together with a substantial improvement in cardiac function.

Bone Marrow Transplantation↗
PubMed1996

Mucormycosis after bone marrow transplantation: report of four cases in thalassemia and review of the literature.

We report four cases of mucormycosis that occurred among 711 patients who underwent BMT for thalassemia, and review 18 additional cases among BMT recipients that were reported in the English-language literature. All these patients were polytransfused and were in advanced phase of disease with severe acquired hemochromatosis. The sites of infection were sinonasal, rhinocerebral-pulmonary, pulmonary and pulmonary-central nervous system. Mucormycosis was the primary cause of death in three of four patients. Two infections were detected within the first 100 days after BMT. Only one of the four patients had partial resolution of sinonasal mucormycosis following aggressive antifungal therapy combined with hyperbaric oxygen treatment.

Adolescent↗
PubMed1996

CsA-associated neurotoxicity and ineffective prophylaxis with clonazepam in patients transplanted for thalassemia major: analysis of risk factors.

Cyclosporin A (CsA) has been shown to be useful in the prophylaxis of acute graft-versus-host-disease (GVHD). However, this immunosuppressive agent produces multiple side-effects including nephrotoxicity, hypertension, hypertricosis, gum hyperplasia, infections, and neurotoxicity. We report a retrospective analysis of neurotoxicity in 625 recipients transplanted for thalassemia and given CsA as part of GVHD prophylaxis. Neurotoxicity consisted in mental status changes, tremor, headache (grade 1), visual disturbance and cortical blindness (grade 2) and seizures and coma (grade 3). The overall toxicity was 28.8% and the incidence of convulsions was 10.1%. Neurological findings were reversible after temporary reduction or discontinuation of CsA. Class 3 patients, when prepared with protocol 6 (Bu 14 + Cy 200 and CsA for GVHD) or when they developed acute GVHD, had the highest risk of convulsions. Age, sex, different conditioning regimens, different anticonvulsive prophylaxis, liver damage due to iron-overload and/or to chronic inflammation did not influence the occurrence of CsA-related CNS toxicity. The occurrence of acute GVHD with concomitant use of high-dose corticosteroids is the single significant predisposing factor in the occurrence of convulsions. Grades 1 and 2 of neurotoxicity occurred earlier and were not influenced even by acute GVHD.

Acute Disease↗
PubMed1995

Social and geographical inequalities in prenatal care in Italy.

The purpose of this study was to find out possible differences in prenatal diagnosis (PD) by amniocentesis or chorionic villus sampling and ultrasound examination before 16 weeks' gestation (early ultrasound examination, EUE), according to area of residence and level of education of the mothers. Data come from a multicentre study in Italy involving 43 maternity hospitals (Mercurio project) and were collected through structured interviews a few days after delivery. Study subjects were 1541 mothers of single non-malformed infants born between April 1992 and March 1994. Overall, the incidence of PD and EUE was 5.8 and 80 per cent, respectively. After adjustment for maternal age and other possible confounders, the odds ratios for PD were 2.19 (95 per cent confidence interval (CI) = 1.26-3.81) when women residing in the northern regions were compared with those residing in the southern regions, and 2.06 (95 per cent CI = 1.12-3.79) and 4.08 (95 per cent CI = 1.97-8.42) when women with medium and high levels of education were compared with those with low level. For EUE, the odds ratios were 1.77 (95 per cent CI = 1.32-2.36) and 2.88 (95 per cent CI = 1.56-5.29) when comparing women with medium and high levels of education with those with low level. No relationship was found between area of residence and EUE. These geographical and social inequalities in prenatal care in Italy should be taken into account for the organization, delivery, and evaluation of pregnancy services in the coming years.

Adult↗
PubMed1995

Ultrasonography in pregnancy and fetal abnormalities: screening or diagnostic test? IPIMC 1986-1990 register data. Indagine Policentrica Italiana sulle Malformazioni Congenite.

The aim of the present study was to assess the sensitivity of ultrasound diagnosis used as a screening test in detecting major congenital anomalies in the prenatal period in a large nation-based multicentre setting. Data from the IPIMC register were collected in the period 1986-1990. One hundred and thirty-five hospitals, located in 17 out of the 20 regions in Italy, participated in the register. Study cases were 3479 infants with major congenital anomalies diagnosed at birth or in the first week of life. Subjects with chromosomal anomalies or multiple defects were excluded. The sensitivity of ultrasound prenatal diagnosis was 49.5 per cent for central nervous system anomalies, 3.8 per cent for congenital heart diseases, 17.1 per cent for gastrointestinal tract defects, 46.6 per cent for abdominal wall defects, 74.8 per cent for urinary tract anomalies, and 22.9 per cent for skeletal abnormalities. The detection rate for diaphragmatic hernia was 24.2 per cent. Overall, only 18 per cent of the defects diagnosed in utero were detected before 24 weeks' gestation. The sensitivity of prenatal diagnosis was 30.1 and 19.0 per cent in the northern, central, and southern regions, respectively. In light of its low sensitivity, ultrasonography as a screening test in the general population should be abandoned, although some improvement in its performance should be expected following adequate training of the ultrasound staff and the use of good technical equipment.

Abdominal Muscles↗
PubMed1995

Needle liver biopsy in thalassaemia: analyses of diagnostic accuracy and safety in 1184 consecutive biopsies.

We report the reliability and safety of percutaneous liver biopsy in the evaluation of hepatic iron loading and histology in patients with homozygous beta-thalassaemia prior to and in serial biopsies following allogeneic bone marrow transplantation for this disorder. 501 thalassaemic patients aged 11 +/- 4.5 years (range 1-32 years) underwent 1184 consecutive percutaneous liver biopsies without ultrasound guidance. Overall, 81% of biopsies were evaluable for histological examination and grading of iron. The adequacy of liver biopsy specimens increased with patient age: evaluable specimens were obtained in 73% of patients < 5 years of age and in 86% of samples in patients aged > 15 years. The degree of iron overload and fibrosis in each biopsy was reported separately by at least two pathologists who did not know the clinical status of each patient. In 103 biopsies, iron grade by light microscopy corresponded to an iron concentration varying between a mean of 32.46 +/- 14 mumol/g dry weight liver tissue for iron stores graded by light microscopy as absent to 417.6 +/- 150 mumol/g dry weight liver tissue for stores graded as severe. The fibrosis score of multiple samples of liver obtained at autopsy within 100 d of the percutaneous biopsy in 41 patients who died following BMT correlated perfectly with that of the first sample in > 60% biopsies; in most of the discordant cases fibrosis had been underestimated in the percutaneous biopsy. Liver biopsy demonstrated evidence of chronic hepatitis in 30% of patients with normal transaminase and in 57% of patients with transaminase within twice the normal range. Liver biopsy was complicated in six patients (0.5%) by haemoperitoneum, periocholecystic haematoma, kidney haematoma, or bile peritonitis; no complication was fatal. These data demonstrate that percutaneous liver biopsy provides reliable information regarding liver iron and histology in homozygous beta-thalassaemia with an extremely low risk of complications.

Adolescent↗
PubMed1995

Desferrioxamine therapy accelerates clearance of iron deposits after bone marrow transplantation for thalassaemia.

We treated 18 heavily iron-loaded patients who had become ex-thalassaemics after bone marrow transplantation with subcutaneous desferrioxamine therapy for 5-20 months. As determined using serum ferritin concentration, transferrin saturation and stainable liver iron obtained in follow-up biopsies, marked decreases in body iron stores were observed with this regimen. Moreover, the liver function tests demonstrate a trend to normalization in all cases. Local skin reactions to desferrioxamine were the only toxicities observed. We conclude that pharmacological iron chelation is a safe and effective therapy in the reduction of iron deposits in this clinical situation; it therefore represents a valid alternative to phlebotomy in selected patients.

Adolescent↗
PubMed1995

Suprapubic cystotomy as treatment for severe hemorrhagic cystitis after bone marrow transplantation.

We analyzed the success of suprapubic cystotomy in patients with severe hemorrhagic cystitis after bone marrow transplantation. Seventy-three out of 963 patients developed severe hemorrhagic cystitis which resulted in urinary tract obstruction after high-dose cytoreductive therapy. Eleven patients (15%) failed medical treatment and required emergency suprapubic cystotomy. Three of these patients died of other complications prior to resolution of HC. Of the remaining 8 patients who underwent surgery, 4 are alive. The mortality rate was significantly higher in patients who required surgery than in those who responded to medical therapy. Patients whose HC required surgery also had a greater transfusion requirement than those who responded to medical therapy. We conclude that surgical treatment of severe HC should be undertaken only after failure of medical therapy.

Adolescent↗
PubMed1995

Haploidentical bone marrow transplantation from mother to child with advanced leukemia.

Use of the mother as mismatched marrow donor was assessed in 19 children with advanced leukemia. Patients were homogeneous for HLA incompatibility, age, donor, and conditioning regimen, and stage of disease. All received busulfan and cytoxan, combined with unmodified donor marrow, ALG given before and after transplant, and short MTX and cyclosporine as GVHD prophylaxis. Survival, LFS, and relapse respectively were 26, 26, and 33%. Incidence of overall and severe acute GVHD was 58 and 32%, respectively. Four patients had failure of engraftment, and two of these are alive with autologous reconstitution in complete remission. Probability of rejection was 21%. Results of haploidentical transplants were compared with those of children with advanced leukemia treated at the same institution, who received marrow from HLA-identical siblings. The probability of long-term leukemia-free survival was similar in the two groups. We thus propose using the mother as an alternative marrow donor in children with advanced leukemia.

Adolescent↗
PubMed1994

Hepatitis C virus infection in thalassemia patients undergoing allogeneic bone marrow transplantation.

Ninety-eight patients with homozygous-beta thalassemia who had undergone allogeneic bone marrow transplantation (BMT) between May 1990 and March 1992 were tested for hepatitis C antibodies (anti-HCV) before and after BMT. Anti-HCV positivity was detected in 50 of the 98 patients (51%) before BMT. Seroconversion was demonstrated in seven of the 40 evaluable seronegative patients. In four cases it was probably due to the different sensitivity of first and second generation ELISA. Of the 46 evaluable seropositive patients 4 had transient and 5 persistent negativity for HCV antibodies after BMT. The high prevalence of anti-HCV positivity in thalassemic patients is related to the continuous requirement for blood transfusions. We found a strong correlation between biochemical and histological evidence of liver damage and anti-HCV positive status in multi-transfused patients. In our experience HCV hepatitis does not influence the outcome of BMT.

Adolescent↗
PubMed1994

Cardiac tamponade in thalassemia.

We report a case of acute cardiac tamponade without concurrent myocardial disease occurring in a thalassemia patient early after bone marrow transplantation. The pericardial effusion was preceded by an episode of junctional tachycardia. Repeated evaluation by echocardiography was done shortly after the patient developed the arrhythmia and permitted a detailed, timed observation of the event and description of the symptoms.

Bone Marrow Transplantation↗
PubMed1994

Allogeneic marrow transplantation in patients with chronic myeloid leukemia in chronic phase following preparation with busulfan and cyclophosphamide.

Thirty-four patients with chronic myelogenous leukemia in chronic phase were treated with busulfan 16 mg/kg and cyclophosphamide 120 or 200 mg/kg before allogeneic bone marrow transplantation from an HLA-identical sibling. Cyclosporine, methotrexate and prednisone were used for graft-versus-host disease (GVHD) prophylaxis. The actuarial probabilities of survival and relapse-free survival at 82 months were 71%. With a maximum follow-up of 2471 days, none of the patient experienced hematologic or clinical relapse. In one patient reappearance of host cells was documented 180 days post-transplant which disappeared 277 days post-transplant and the patient is in complete hematological and cytogenetic remission 5 years after the transplant. The probability of transplant-related mortality was 29% while the probability of moderate to severe acute graft-versus-host disease was 38%. This study indicates that busulfan and cyclophosphamide are a good conditioning regimen for marrow transplantation in patients with chronic myeloid leukemia in chronic phase.

Adolescent↗
PubMed1994

Bone marrow transplantation for thalassemia. Experience in Pesaro, Italy.

PURPOSE: We reviewed the results of transplanting allogeneic marrow from HLA-identical donors in patients with beta-thalassemia. Among the 484 consecutive patients who have received transplants since 1981, survival and disease-free survival rates leveled off at approximately 1 year after transplantation, at 82 and 75%, respectively. PATIENTS AND METHODS: Clinical characteristics of patients before transplant have been studied to determine their impact on survival, disease-free survival, and graft rejection. By multivariate analysis, portal fibrosis, hepatomegaly, and a history of inadequate chelation therapy were identified as risk factors. The patients were then divided into three classes of risk. RESULTS: The rate of prolonged disease-free survival was 98% and 87% for class 1 and class 2 patients. This rate of disease-free survival is 70% with the use of our last conditioning protocol for class 3 patients. Older patients (17-32 years) have a 79% probability of prolonged disease-free survival. CONCLUSIONS: We conclude that for patients with thalassemia major, transplantation of bone marrow from a human leukocyte antigen-identical donor offers a high probability of disease-free survival, particularly for those patients in early stages of their disease.

Adolescent↗
PubMed1993

Fate of iron stores in thalassaemia after bone-marrow transplantation.

After successful bone-marrow transplantation (BMT) in thalassaemia, the individual acquires the pattern of globin synthesis of the donor. We call such an individual "ex-thalassaemic after BMT", a term that underscores the cure of the genetic defect but maintenance of residual signs of organ damage due to iron overload and dysfunction acquired during the pretransplant years. We have analysed the extent and fate of tissue iron overload in 151 ex-thalassaemic patients after BMT, according to the risk factors of hepatomegaly, hepatic portal fibrosis, and inadequate chelation therapy. Serum ferritin concentrations decreased and unbound iron binding capacity (UIBC) increased slowly during the years after the transplant. When analysed according to risk group (assigned at the time of the transplant), ferritin and UIBC returned within the normal ranges in only the low-risk group (without hepatomegaly or portal fibrosis, and with adequate chelation pre-BMT). Ferritin and UIBC were still abnormal 7 years after the transplant in the moderate-risk group (those with one or two risk factors) and highly abnormal in the high-risk group (all three risk factors) indicating persistence of, respectively, moderate and severe iron overload at the time of transplant. In ex-thalassaemic patients who were studied before and yearly after the transplant the extent of haemosiderosis, as judged by staining of liver biopsy samples, decreased during the years after transplant. The degree of iron deposition and rate of post-BMT linear growth seem to influence rate of post-BMT decrease in tissue iron overload in different risk groups at the time of BMT.

Adolescent↗
PubMed1993

Marrow transplantation in patients with thalassemia responsive to iron chelation therapy.

BACKGROUND: Patients with homozygous beta-thalassemia, who have a good prognosis during treatment with conventional therapy, appear to have an especially high probability of hematologic cure with bone marrow transplantation, although the morbidity and mortality associated with such treatment are not established. METHODS: The records of all patients with thalassemia who received bone marrow transplants from HLA-identical donors in Pesaro, Italy, were examined from October 1982 through May 1992. Detailed evaluation of the outcome was conducted in the 89 patients identified as being in class 1 according to the Pesaro classification, in which hepatomegaly, portal fibrosis, and the inadequacy of iron chelation therapy are considered independent risk factors, and the patients are classified as being in class 1 if none of these factors are present, class 2 if one or two of the factors are present, and class 3 if all three factors are present. Sixty-four of the patients had been prepared for transplantation with a drug regimen in current use that includes busulfan and cyclophosphamide followed by cyclosporine as prophylaxis against acute graft-versus-host disease (protocol 6). RESULTS: There were seven deaths, all within 101 days of transplantation. Two of the 64 patients treated according to protocol 6 died. The probabilities of survival, rejection-free survival, death from causes unrelated to rejection, and rejection were 0.92, 0.85, 0.06, and 0.08, respectively, in the total group and 0.97, 0.93, 0.03, and 0.04 in the 64 patients treated according to protocol 6. Preliminary evidence suggests that there was useful unloading of tissue iron deposits. CONCLUSIONS: The high probability of cure with little early or late morbidity and mortality suggests that patients with class 1 thalassemia who have HLA-identical donors available should be treated by bone marrow transplantation. However, this was not a controlled trial, so we cannot directly compare the outcome with that of conventional treatment.

Adolescent↗