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R Colella

Publications and source records attributed to R Colella.

At least 37 records · Page 2Linked to original sources
PubMed1993

Langerhans cell histiocytosis in childhood: results from the Italian Cooperative AIEOP-CNR-H.X '83 study.

Ninety patients with biopsy-proven Langerhans cell histiocytosis (LCH) were enrolled from June, 1983, to December, 1988, in the multicenter AIEOP-CNR-H.X. '83 study. They were divided into two groups: poor prognosis (PP), comprising 11 children with organ dysfunction (OD), and good prognosis (GP), made up of 79 patients without OD. Eighty-four patients were evaluable for treatment results. Among GP patients, 16 with a single lesion received only local treatment, while 59 entered a clinical trial of immunotherapy and/or monochemotherapy with vinblastine (VBL). Nonresponders, sequentially received doxorubicin (ADM) and then etoposide (VP16). PP patients were treated with 4 week cycles of vincristine, ADM, cyclophosphamide, and prednisone for nine courses. The overall survival was 92.8% (100% for GP patients and 45.5% for PP patients) at 48 months. The complete response (CR) rates for immunotherapy, VBL, ADM, and VP16 were 10%, 62.9%, 42.8%, and 88.2%, respectively. Two of the 11 PP patients had a CR (18.2%), while six died and three are still alive with recurrent disease. The overall incidence of disease-related disabilities was 47.7%, while that of diabetes insipidus was 20%. Monochemotherapy is probably adequate in GP patients, while more effective treatments are needed for PP patients.

Adolescent↗
PubMed1993

Outcome after cessation of therapy in childhood acute lymphoblastic leukaemia. The Associazione Italiana Ematologia ed Oncologia Pediatrica (AIEOP).

A total of 2192 children with acute lymphoblastic leukaemia who had reached cessation of therapy in complete remission were followed for a median time of 52 months after treatment suspension. Of the 485 relapses observed, 62.3% occurred in the first year off therapy and 68.9% involved the bone marrow. Eight relapses were reported more than 5 years (62-143 months) after treatment withdrawal. Males fared worse than females consistently, experiencing 1.5 times more relapses (P < 0.0001). Thirteen patients died in continuous complete remission, 5 because of non-neoplastic central nervous system complications. There were 11 second solid malignancies, 8 of them in the central nervous system; 9 subjects presented an haematopoietic malignancy after ALL. The projected event-free survival at 8 years is 73%. Twenty-two of the 171 young adults (age > 20 years) were married and 16 have had 21 healthy children. Twenty-four per cent of patients experienced an unfavourable event. Relapses accounted for 93% of failures. Central nervous system late effects and second malignancies were the major causes of non-leukaemic morbidity and mortality.

Adolescent↗
PubMed1992

Increased expression of cathepsins L and B and decreased activity of their inhibitors in metastatic, ras-transformed NIH 3T3 cells.

We previously found that the T24 Ha-ras oncogene induces metastatic ability in NIH 3T3 cells and that this change depends on expression of the ras oncogene. As part of our studies on mechanisms by which ras may induce metastasis, we investigated expression and activity of two cysteine proteinases, cathepsin L (major excreted protein) and cathepsin B, as well as cysteine proteinase inhibitor activity, in ras-transformed NIH 3T3 cells. In a series of cel lines that expressed differing amounts of ras, we found a good correlation between levels of ras expression and cathepsin L expression (r = 0.80). There was also a good correlation between secreted procathepsin L protein levels and experimental metastatic ability (r = 0.88). We found a similar but less strong association between cathepsin B levels and metastatic ability in these cells (r = 0.76 and r = 0.72 for 2.2-kb and 4.1-kb transcripts, respectively). Functional cathepsin L plus B activity (both secreted and cell-associated) was found to be higher in ras-transformed cells and was dependent on cell confluency in culture. Coupled with increased expression and activity of cysteine proteinases, ras-transformed NIH 3T3 cells showed reduced cysteine proteinase inhibitor activity. We conclude that the balance between expression of cysteine proteinases and their inhibitors may be coregulated by ras expression. Our results suggest that ras-induced increases in production of degradative enzymes such as cathepsins L and B, along with decreased activities of their inhibitors, may contribute to the increased malignant properties of ras-transformed NIH 3T3 cells.

3T3 Cells↗
PubMed1991

Steady-state cystatin mRNA levels in chicken tissues in response to estrogen.

A full length cDNA clone coding for the cysteine proteinase inhibitor, chicken cystatin, was used to hybridize to RNA extracted from various tissues of the hen during several well defined stages of the 25 h ovulatory cycle. Measurements were made immediately after egg-laying (ovulation), 6 h prior to ovulation (estrogen surge), and 16 h prior to ovulation (minimum estrogen levels). Cystatin was expressed in all tissues examined, being most abundant in lung, followed by brain, heart, oviduct, pectoral muscle, and liver in decreasing order. There were no significant differences in the cystatin mRNA levels at the three different time points for any of the tissues examined, and no differences in the 0.95 kb size of the message. Pharmacological doses of estradiol administered to immature female chickens decreased the steady-state cystatin mRNA levels as analyzed by Northern blots. There were no differences in the pattern of expression in the different tissues between rooster and hens. Our data suggest that estrogen has no direct effect on the regulation of cystatin expression in chicken tissues.

Animals↗
PubMed1990

Idarubicin and high-dose cytarabine in the treatment of refractory and relapsed acute lymphoblastic leukemia.

Between August 1985 and April 1989, 88 patients (31 children and 57 adults) with refractory of relapsed acute lymphoblastic leukemia (ALL) were treated in a cooperative Italian trial by an induction schedule of high-dose Cytarabine (HDAra-C) plus Idarubicin (IDA). Complete remission (CR) was achieved in 52 of the 88 patients (59%); 23 patients (26%) did not respond to treatment and 13 (15%) died during induction. The CR rate was significantly affected by the WBC count at the beginning of treatment and by the duration of first CR of the patients treated at first relapse. All of the patients experienced profound myelosuppression; the median time to recovery to neutrophils greater than 0.5 x 10(9)/l was 15 days (range 4-40), and 14 days (range 3-50) to platelets greater than 50 x 10(9)/l. The most common non-hematologic side effects observed were nausea and vomiting (51%), mucositis (40%) and diarrhea (23%). Twenty-one of the 52 patients who achieved CR underwent bone marrow transplantation (BMT), 16 autologous and 5 allogeneic. Eleven patients relapsed at a median of 4 months (range 1-31) after the transplantation, and three patients died while in CR. Seven patients have been in continuous CR (CCR) for a median of 36 months (range 26-42 months). Thirty-one patients were not entered in the BMT program: for two adults it was too early, three adults died in CR and 25 patients relapsed at a median of four months (range 1-25). Only one adult is still in CCR at 33 months.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1989

Chicken egg white cystatin. Molecular cloning, nucleotide sequence, and tissue distribution.

A lambda gt11 chicken oviduct cDNA library was screened with a mixed synthetic oligonucleotide corresponding to amino acid residues 81-90 of chicken egg white cystatin, a cysteine proteinase inhibitor. Two initial cDNA clones of 367 and 431 bases were isolated. Both clones contained coding sequences for cystatin from amino acid residue 82 to the carboxyl end plus 3'-untranslated region and a poly(A)+ tail. The two clones utilized different polyadenylation signals located 55 nucleotides apart. Further screening of the library yielded a full-length cystatin cDNA. Sequence analysis indicated that cystatin contains an NH2-terminal extension of 23 amino acids which is probably a signal sequence. The cystatin cDNA hybridized to an mRNA of approximately 0.95 kilobase and was present in varying amounts in all chicken tissues examined. The highest concentration was found in the lung. Gizzard, brain, and heart contained lesser amounts of cystatin mRNA but considerably higher than oviduct. Among a limited number of embryonic tissues examined, significantly higher levels of the mRNA were found in liver and heart tissues when compared with the corresponding adult tissues. These results suggested that the expression of the chicken cystatin gene is tissue-dependent and under developmental control.

Amino Acid Sequence↗
PubMed1989

Angioimmunoblastic lymphadenopathy with dysproteinemia: report of a case in infancy with review of literature.

A case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) in infancy is reported. The disease had a mild onset with generalized lymphadenopathy, hepatosplenomegaly, thrombocytopenia, polyclonal hypergammaglobulinemia, and T-cell deficiency. The AILD course lasted more than 100 months, alternating clinical remission to recurrent relapses. Hepatitis B viral infection suddenly evolving to hepatic failure was the cause of death. From a rapid survey of the present knowledge, the nosology, immunological features, and therapy of AILD are discussed and a possible presumptive pathogenetic pathway is proposed.

Blood Protein Disorders↗
PubMed1988

Etoposide in recurrent childhood Langerhans' cell histiocytosis: an Italian cooperative study.

Eighteen evaluable children with recurrent Langerhans' cell histiocytosis (LCH) which was resistant to standard therapy, were treated with etoposide (VP 16-213), 200 mg/m2/day for 3 days every 3 weeks, to study the efficacy and toxicity of this drug. Complete and partial responses were demonstrated in 15 patients (83.3%). Only one of the 12 children achieving a complete remission has relapsed. No dose-limiting major toxicities were registered. Although etoposide might be an effective treatment in recurrent LCH which needs a chemotherapeutic approach, it is emphasized that this drug must be used carefully.

Child↗
PubMed1988

Correlation between cranial computed tomographic scans at diagnosis in children with acute lymphoblastic leukaemia and central nervous system relapse.

145 children with acute lymphoblastic leukaemia (ALL) were evaluated over a period of 3 years in a multicentre study in which serial cranial computed tomographic (CT) scans of the brain were done. All patients were symptom-free. CT scans were graded as normal, borderline (slight or moderate cerebral atrophy), or pathological (severe cerebral atrophy). 62% (90/145) of children had CT scan abnormalities at diagnosis. After a median follow-up of 24 months (range 6-36) 12 of 108 evaluable patients had central nervous system (CNS) relapses (6 isolated relapses and 6 combined with relapse at another site). All patients with CNS relapse had an abnormal CT scan at diagnosis (8 pathological and 4 borderline). No relapses were observed among the 42 patients with a normal cranial CT scan at diagnosis. A significantly higher proportion of severe cerebral atrophy, both following CNS prophylaxis and after the discontinuation of treatment, was found among patients with a borderline CT scan at diagnosis than among patients with a normal CT scan at diagnosis. Thus an abnormal cranial CT scan at diagnosis in children with ALL seems to have prognostic significance.

Adolescent↗
PubMed1987

Undifferentiated (embryonal) sarcoma of the liver in childhood: results of a retrospective Italian study.

The clinical features and the treatment of undifferentiated (embryonal) sarcoma of the liver in 8 patients younger than 19 years old were analyzed. All these cases were registered in the retrospective multicentric study on childhood malignant tumors of the liver, conducted between 1983 and 1985 by the Italian Association of Pediatric Hematology Oncology. The age of the patients ranged from 94 to 190 months (median = 113.5 months); all children were males. An abdominal mass was the common presenting features. Abnormalities in hemogram and common liver tests were rarely reported. Angiography revealed various degrees of vascularization in these tumors. Two patients achieved a surgical complete remission (CR) at diagnosis; one patient achieved surgical CR after primary chemotherapy with vincristine, adriamycin, cyclophosphamide and 5-fluorouracil, which reduced the tumor volume and permitted surgical resection. Two of these patients are still in CR at 14 and 60 months after diagnosis; the third patient died of liver failure without evidence of recurrence 6 months after diagnosis. All of the other patients, who never achieved CR, died of disease. One was lost to follow-up, and one surgical death occurred. Reports of childhood undifferentiated sarcoma are reviewed.

Child↗
PubMed1986

mRNA levels of cathepsins B and D during myogenesis.

Muscle development is characterized by the fusion of myoblasts to form myotubes and the co-ordinate expression of muscle-specific proteins such as actin, myosin and creatine phosphokinase. Our laboratory has been involved in the study of the role of lysosomal proteinases, namely, cathepsins B, H and L and the endogenous cysteine proteinase inhibitor, cystatin, during muscle differentiation in vitro. Specific activities of the cysteine proteinase in chicken primary cultures and a number of rat myogenic lines increased with the degree of myotube formation. This has suggested that lysosomal proteinases play an important role in myogenesis. We have measured the mRNA levels of two of the lysosomal enzymes, cathepsins B and D. This is advantageous because the presence of the endogenous inhibitor, cystatin, masks the levels of the specific activities of the cysteine proteinases present in the cell. RNA was extracted from developing muscle at three stages of development: proliferating myoblasts, confluent cells, and myotubes. Hybridization of RNA extracted from the L6 myogenic cell line with cathepsin B cDNA showed an increase in the level of cathepsin B mRNA. However, in the L8 rat myogenic line the level decreased after fusion. Cathepsin D mRNA levels remained constant throughout differentiation of the L8 cells. This paper also reports on the characterization of lysosomal proteinases of a newly obtained mouse myogenic line, C2.

Animals↗