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R Pasquini

Publications and source records attributed to R Pasquini.

At least 37 records · Page 2Linked to original sources
PubMed2001

Amyloidotic muscle pseudohypertrophy: case report.

The authors report one case of amyloidosis associated with muscular pseudohypertrophy in a 46-year-old woman, who developed weakness, macroglossia and muscle hypertrophy associated with primary systemic amyloidosis. Electromyography showed a myopathic pattern and bilateral carpal tunnel syndrome. The muscle biopsy presented with a type I and II fiber hypertrophy and infiltration of amyloid material in the interstitious space and artery walls. She underwent bone marrow transplantation with stabilization and subjective improvement of the clinical picture.

Amyloidosis↗
PubMed2001

Reversible posterior leucoencephalopathy syndrome associated with bone marrow transplantation.

Reversible posterior leucoencephalopathy syndrome (RPLS) has previously been described in patients who have renal insufficiency, eclampsia, hypertensive encephalopathy and patients receiving immunosuppressive therapy. The mechanism by which immunosuppressive agents can cause this syndrome is not clear, but it is probably related with cytotoxic effects of these agents on the vascular endothelium. We report eight patients who received cyclosporine A (CSA) after allogeneic bone marrow transplantation or as treatment for severe aplastic anemia (SSA) who developed posterior leucoencephalopathy. The most common signs and symptoms were seizures and headache. Neurological dysfunction occurred preceded by or concomitant with high blood pressure and some degree of acute renal failure in six patients. Computerized tomography studies showed low-density white matter lesions involving the posterior areas of cerebral hemispheres. Symptoms and neuroimaging abnormalities were reversible and improvement occurred in all patients when given lower doses of CSA or when the drug was withdrawn. RPLS may be considered an expression of CSA neurotoxicity.

Adolescent↗
PubMed2001

Sister chromatid exchanges and micronuclei in lymphocytes of operating room personnel occupationally exposed to enfluorane and nitrous oxide.

The objective of this article is to assess whether occupational exposure to anesthetics increases genotoxic risk. We investigated two cytogenetic biomarkers, sister chromatid exchanges (SCE) and micronuclei (MN), in the peripheral blood lymphocytes of 46 anesthesiologists (24 men), working in operating rooms and mostly exposed to enfluorane and nitrous oxide, and 66 controls (35 men), not exposed to chemicals and living in the same area. Contrary to what was expected, a lower frequency of SCE was found in male anesthesiologists than in controls. Smoking status was found to be positively associated with SCE frequency in each group, while no relation to age was evident. On the contrary, MN frequency was significantly higher in female, but not male, anesthesiologists than in controls. Age and smoking status did not modify the association. No relationship between MN frequency and duration of employment was found in anesthesiologists. Smoking status and mean number of cigarettes smoked per day in smokers were not associated with MN frequency in either anesthesiologists or in controls. MN analysis seems to be a sensitive index of possible genotoxic effects of occupational exposure to anesthesiologists, and women appear to be more susceptible to these effects than men.

Adult↗
PubMed2000

Outcome of 69 allogeneic stem cell transplantations for Fanconi anemia using HLA-matched unrelated donors: a study on behalf of the European Group for Blood and Marrow Transplantation.

Allogeneic stem cell transplantation is the only treatment that can restore a normal hematopoiesis in Fanconi anemia (FA). In this retrospective multicenter study, we analyzed the results of this approach using HLA-matched unrelated bone marrow donors, and tried to identify covariates predicting the outcome of the transplant. From January 1985 to June 1998, 69 FA patients were transplanted with unrelated HLA-matched donors. Patients' characteristics before and after transplant were provided by the European group blood and marrow transplant registry and were analyzed in collaboration with the European Fanconi Anemia Registry. The 3-year probability of survival was 33%. Extensive malformations, a positive recipient cytomegalovirus serology, the use of androgens before transplant, and female donors were associated with a worse outcome. Primary graft failures were observed more frequently when female donors were used, mainly because the grafts contained lower nucleated cell doses per kilogram of recipient body weight compared with grafts coming from male donors. The probability of grade III-IV acute graft-versus-host disease (GVHD) was 34%. Elevated serum alanine/aspartate transaminases before transplantation; limb, urogenital tract, or nephrologic malformations; and non-T-cell-depleted grafts were predictors of severe acute GVHD. This study shows the dramatic impact of preexisting congenital malformations on the outcome of FA patients transplanted with HLA-matched unrelated donors. If the use of T-cell depletion has led to a dramatic reduction of acute GVHD incidence, no significant outcome improvement was observed with this approach, mainly because of an increased risk of graft failure. (Blood. 2000;95:422-429)

Adolescent↗
PubMed2000

In vitro genotoxicity of terbutryn evaluated by the alkaline single-cell microgel-electrophoresis "comet" assay.

Terbutryn, a s-triazine herbicide, is extensively used in agriculture as a selective pre- and postemergence control agent for most grasses and many annual broadleaf weeds in cereal and legume fields, and under fruit trees. Terbutryn was reported to degrade slowly, with half-lives of 240 and 180 days in pond and river sediment, respectively. The tendency of this herbicide to move from treated soils to water compartments through water runoff and leaching was demonstrated and residual amounts of terbutryn and its metabolites have been found in drinking water, and industrial food products, long after application. Although this herbicide may be regarded as a contaminant of our environment, only limited and inconsistent data exist concerning its genotoxic properties. In this study, the DNA-damaging ability of the herbicide was evaluated in the alkaline single-cell microgel-electrophoresis ("comet") assay by testing terbutryn in the presence of S9mix (rat liver homogenate containing microsomal enzymes plus cofactors) prepared with liver homogenate from both uninduced (basal) and aroclor 1254-induced rats. DNA damage was recorded in freshly isolated human peripheral blood leukocytes. A statistically significant increase in the extent of primary DNA damage, more pronounced in the absence of S9mix, took place only when terbutryn concentrations were high (100 and 150 microg/ml), in the presence of a concomitant mild cytotoxic effect.

Adult↗
PubMed2000

Neuropathological findings after bone marrow transplantation: an autopsy study of 180 cases.

We prospectively evaluated the neuropathological complications of 180 patients who underwent autopsy studies following bone marrow transplantation (BMT) (177 allogeneic, three autologous). The most frequent underlying disorders included severe aplastic anemia (n = 55), chronic myelogenous leukemia (n = 53), acute myelogenous leukemia (n = 24) and Fanconi anemia (n = 16). There were 114 males and 66 females. Neuropathological findings were detected in 90.55% of the patients. The most frequent findings were subarachnoid hemorrhages (SAH) (n = 57), intraparenchymal hemorrhages (IHP) (n = 49), fungal infections (n = 16), Wernicke's encephalopathy (n = 10), microglial nodular encephalopathy (n = 10) and neurotoxoplasmosis (n = 8). In only 17 patients was the brain within normal limits. Survival time after BMT averaged 5.4 months and the majority of patients died in the first 3 months post BMT (n = 105). Central nervous system (CNS) pathology was the main cause of death in 17% of the patients (n = 31), with a predominance of IHP in this particular group. Furthermore, the survival time of these patients who died of CNS causes (96.3 days) was almost half of the survival time of those who died of extra-cerebral causes (177.8 days) (P = 0.0162). IHP (70. 96 vs27.22%) (P < 0.001), fungal infections (25.8 vs 8.88%) (P < 0. 001) and toxoplasmosis (9.67 vs 4.44%) (P < 0.001) were significantly more frequent in the group of patients who died due to CNS causes than in the control group. The findings of this work provide a possible guide to the possible causes of neurological syndromes following BMT. Bone Marrow Transplantation (2000) 25, 301-307.

Adolescent↗
PubMed2000

Cytomegalovirus as a cause of very late interstitial pneumonia after bone marrow transplantation.

Cytomegalovirus (CMV) infection is an important cause of morbidity and mortality after allogeneic transplant. Interstitial pneumonia (IP) is the most common manifestation of CMV in BMT patients, with a 30-48% mortality rate despite adequate treatment. Most CMV infection occurs in the first 100 days. However, prolonged ganciclovir (GCV) prophylaxis has favored the occurrence of late CMV IP, probably by inhibition of the development of CMV-specific T cell lymphocyte responses. We report the case of a patient treated with an allogeneic BMT who received pre-emptive GCV until day +100 because of CMV-positive antigenemia. He developed a CMV IP on day +811 post BMT, which responded to treatment. We intend to alert clinicians that even at long-term (>1 year) post-BMT, CMV is a possible cause of IP in high-risk patients.

Adult↗
PubMed2000

Central nervous system infections following bone marrow transplantation: an autopsy report of 27 cases.

The authors retrospectively assess the autopsy findings of central nervous system (CNS) infections in marrow transplant recipients. From July 1987 to June 1998, 845 patients at our institution were submitted to bone marrow transplantation (BMT). The CNS of 180 patients was studied through autopsy and these patients had their medical records reviewed. Twenty-seven (15%) patients presented brain parenchyma infection. Fungi were isolated in approximately 60% of the cases. Mean survival time was 153 days (0-1,264 days) and the majority of the patients died during the first 3 months after BMT (18 cases; 67%). Aspergillus sp. were the most prevalent fungi (approximately 30%), followed by Candida sp. infection (approximately 18%). There was one case of Fusarium sp. infection and two cases of unidentified fungus. All patients with fungal infections had documented involvement at widespread sites. Toxoplasma gondii encephalitis was demonstrated in 8 patents (approximately 30%). Bacterial abscesses were responsible for approximately 11% of the findings. Eleven (41%) of the 27 patients died secondary to cerebral causes. These results show that infectious compromise of the CNS following BMT is a highly fatal event, caused mainly by fungi and T. gondii. Furthermore, they provide a likely guide to the possible causes of brain abscesses following BMT.

Adolescent↗
PubMed2000

Long-term outcome of 25 children and adolescents with severe aplastic anemia treated with antithymocyte globulin.

Severe aplastic anemia (SAA) is probably an immune-mediated disorder, and immunosuppressive therapy is recommended for patients with no available donor for bone marrow transplant. Between October 1984 and November 1987, 25 consecutive children and adolescents with SAA with no HLA-compatible marrow donor received equine antithymocyte globulin (ATG) (15 mg kg-1 day-1) for 10 days. The patients were evaluated 6 weeks, 6 months, and 12 months after starting ATG treatment. Thereafter, patients were evaluated yearly until July 1998. Median age was 10 years (range, 1.5-20 years), granulocyte counts on referral ranged from 0.032 to 1.4 x 10(9)/l (median 0.256 x 10(9)/l), and 12 patients had granulocyte counts <0.2 x 10(9)/l. At a median follow-up of 9.6 years (range, 8.6-11.8 years), 10 patients (40%) remained alive with good marrow function. No morphologic evidence of hematological clonal disorders has been observed, although two patients probably have acquired clonal chromosomal abnormalities (trisomy 8 and del(6)q21, respectively). Responses to ATG were observed between 6 weeks and 6 months from the start of treatment in 60% of evaluable patients. The response rate was not different in patients whose granulocyte count at diagnosis was <0.2 x 10(9)/l, or in those who were <10 years of age. This study supports the view that, when compared with supportive measures, ATG is an effective treatment for children or adolescents with SAA. Although these results are inferior to those reported for marrow transplantation or more intensive immunosuppressive regimens, these patients who responded to ATG are long-term survivors with stable peripheral blood counts and a low rate of relapse.

Adolescent↗
PubMed2000

[Obstructive syndrome caused by trichobezoars: historical disease or disease still current? Description of a case in adolescence].

Trichobezoars are masses of entangled material, found in the stomach and intestines, composed of hair ingested by the patient. When the mass grows, symptoms of intestinal occlusion can appear. Trichobezoars in pediatrics are usually found in adolescent females presenting personality disorders and trichophagia. This work describes a case of trichobezoar diagnosed in a 13 year-old girl, wearing a brace for serious scoliosis but absolutely normal from the emotional and psychical point of view, with normal scholastic and familiar situation. Already hospitalized three months before for pneumonia from mycoplasma, the girl comes to our observation for the appearance of vomiting and constipation. The clinical examination reveal an epigastric mass as big as an orange. General conditions and hematochemical tests are good. Lab tests are performed (abdomen echography and upper abdomen MNR) but is the oesophago-gastroscopy which allows the diagnosis. A big trichobezoar is then surgically removed and the gastroenteric symptoms completely disappear.

Adolescent↗
PubMed2000

[It is not so difficult to carry out simple things properly!].

This report is about the results of the experience we did, since 1996, in the nursing unit of a first level mountain hospital in which we work, where we followed a new childbirth and postpartum assistance protocol aimed to humanizing birth and postpartum, promoting exclusive breastfeeding and reducing childbirth risks. The results show that, since the beginning of our experience, we had an evident reduction of preterm childbirth (newborns with less than 36 weeks of gestational age). We also had a reduction of cesarian sections and of neonatal transferrals in second level neonatal units. Furthermore, we had a progressive growth of exclusive breastfeeding. The report also shows that, since 1996, the newborns discharged from our hospital are exclusively breastfed for a time longer than before and that, at 1 year, 11 percent of our children are still breastfed.

Birthing Centers↗
PubMed2000

[Symptomatic congenital cytomegalovirus infection: report of a clinical case with very severe onset].

This document is about a serious congenital CMV case in a 36 weeks' gestation female newborn with intrauterine growth retardation delivered by cesarean section whose mother was drug addicted. At birth the newborn showed petechiae and bloody blisters all over the body, serious hepatosplenomegaly and microcephaly. Laboratory tests showed thrombocytopeny (platelets count 24.000/mm3) requiring platelets and fresh frozen plasma transfusions during stay in the hospital; echoencephalography showed brain cyst in occipital area, dilated ventricles and calcification in the periventricular area; cardiac echography showed congenital cardiopathy with ventricular and atrial septal defect and patent ductus arteriosus. Urinary presence of CMV-DNA and then CMV-DNA in maternal blood and milk were found. The newborn was given 4 days of endovenous iperimmune immunoglobulin, but the treatment with ganciclovir was impossible for her serious hepatopathy. The newborn was discharged at 45 days and followed monthly with a day hospital program. Now she's 10 months old and she has serious neuromotory problems with left emiparesis and she's following a program of neuromuscular re-education at our hospital.

Cytomegalovirus Infections↗
PubMed1999

Factors associated with outcome after cord blood transplantation in children with acute leukemia. Eurocord-Cord Blood Transplant Group.

We have analyzed factors influencing the outcome of 102 children with acute leukemia given a cord blood transplantation (CBT) and reported to the Eurocord Registry. Seventy patients with acute lymphoblastic and 32 with acute myeloid leukemia were given either a related (n = 42) or an unrelated (n = 60) CBT. Children given CBT during first or second complete remission were considered as belonging to the good-risk group (n = 66), whereas those who received a transplant in a more advanced stage of disease were assigned to the poor-risk group (n = 36). In the related group (RCBT), 12 of 42 patients received transplantation from an HLA-disparate donor, whereas in the unrelated group (UCBT) 54 of 60 received an HLA mismatched CBT. Kaplan-Meier estimates for neutrophil recovery at day 60 were 84% +/- 7% in RCBT and 79 +/- 6% in UCBT (P =.16). In multivariate analysis, the most important factor influencing neutrophil engraftment in UCBT was a nucleated cell dose infused greater than 3.7 x 10(7)/kg (P =.05, relative risk [RR] of 1.85, 95% confidence interval [CI]: 0.98-3.4). The incidence of grade II through IV acute graft-versus-host disease was 41% +/- 8% in the RCBT group and 37% +/- 6% in the UCBT group (P =.59). Kaplan-Meier estimates of 2-year event-free survival (EFS) after RCBT or UCBT were 39% +/- 8% and 30% +/- 7%, respectively (P =.19). In multivariate analysis, the most important factor influencing EFS was disease status at time of transplantation: good-risk patients had a 2-year EFS of 49% +/- 7% as compared to 8% +/- 5% in patients with more advanced disease (P =.0003, RR: 0.40, 95% CI: 0.24 to 0. 65). This was a consequence of both an increased 1-year transplant related mortality and a higher 2-year relapse rate in the poor-risk group (65% +/- 9% and 77% +/- 14%, respectively), as compared with good risk patients (34% +/- 6% and 31% +/- 9%, respectively). These data confirm that allogeneic CBT from either a related or an unrelated donor is a feasible procedure able to cure a significant proportion of children with acute leukemia, especially if transplanted in a favorable phase of disease.

Acute Disease↗
PubMed1999

Primary renal aspergillosis: extremely uncommon presentation in patients treated with bone marrow transplantation.

Invasive aspergillosis affects 3 to 11% of BMT patients with a high mortality rate (60 to 95%). Extra-pulmonary disease is an unusual event, and primary renal aspergillosis is extremely uncommon. A patient with CML treated with BMT, who developed primary renal and subsequently hepatic aspergillosis, is described. Dysfunction of the mucosal barrier secondary to conditioning therapy, was a possible portal of entry for the fungus. Fine needle aspiration was very useful, as is direct microscopic examination of the urine, for diagnosis of the fungal infection. Surgical drainage of the abscess followed by antifungal therapy is the treatment of choice. Unconducive situations, such as refractory thrombocytopenia, are associated with the worst outcome in these patients.

Aspergillosis↗
PubMed1999

Bone marrow transplantation for patients with Fanconi anemia: reduced doses of cyclophosphamide without irradiation as conditioning.

Fanconi anemia (FA), a rare autosomal recessive disease, frequently evolves to bone marrow failure and acute myeloid leukemia, and BMT is the treatment of choice for patients with FA. However, their exquisite hypersensitivity to DNA cross-linking agents is associated with severe complications and several investigators have been looking for the ideal preparatory regimen. We have been involved in a program of progressively decreasing doses of cyclophosphamide (CY) as conditioning therapy, in an attempt to identify the lowest dose of CY capable of maintaining the graft with minimum complications. Here, we describe our experience of allogeneic BMT offered to 16 patients with FA and an HLA-compatible sibling donor, conditioned with 100 mg/kg of CY. The actuarial survival is 88% at approximately 37 months. Mucositis >/= grade II was the most common complication (94%), followed by bacteremias (38%). Veno-occlusive disease and hemorrhagic cystitis did not occur. Sustained engraftment was obtained in 94% of patients, and acute and chronic GVHD was diagnosed in 13% and 7%, respectively. The lowest dose of CY for transplant in FA patients is yet to be determined, but further reductions seem possible.

Adolescent↗
PubMed1999

Antigenotoxic properties of Terminalia arjuna bark extracts.

Compounds possessing antimutagenic properties (polyphenols, tannins, vitamins, etc.) have been identified in fruits, vegetables, spices, and medicinal plants. Terminalia arjuna (Combretaceae), a tropical woody tree occurring throughout India and known locally as Kumbuk, is a medicinal plant rich in tannins and triterpenes that is used extensively in Ayurvedic medicine as a cardiac tonic. The aim of the present collaborative work was to test six solvent extracts from the bark of Terminalia arjuna for antigenotoxic activity using in vitro short-term tests. Terminalia arjuna extracts were obtained by sequential extraction using acetone, methanol, methanol + HCl, chloroform, ethyl acetate, and ethyl ether. The antigenotoxic properties of these extracts were investigated by assessing the inhibition of genotoxicity of the directacting mutagen 4-nitroquinoline-N-oxide (4NQO) using the "comet" assay and the micronucleus (MN) test. Human peripheral blood leukocytes were incubated with different concentrations of the six extracts (from 5 to 100 microg/ mL) and with 4NQO (1 and 2 microg/mL, for the "comet" assay and MN test, respectively). Each extract/4NQO combination was tested twice; in each experiment, positive control (4NQO alone) and negative control (1% DMSO) were set. "Comet" assay results showed that acetone and methanol extracts were highly effective in reducing the DNA damage caused by 4NQO, whereas the acidic methanol, chloroform, ethyl acetate, and ethyl ether extracts showed less marked or no antigenotoxic activity. In the MN test, a decrease in 4NQO genotoxicity was observed by testing this mutagen in the presence of acetone, methanol, chloroform, and ethyl acetate extracts, even though the extent of inhibition was not always statistically significant.

4-Nitroquinoline-1-oxide↗