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Biomedical subjects

R Miniero

Publications and source records attributed to R Miniero.

213 records · Page 12Linked to original sources
PubMedDate not supplied

[The Hbf in Fanconi's anemia (author's transl)].

The Authors studied the HbF in an 8-year-old-girl with Fanconi's anemia. At the admission the patient was anemic (Hb 7,1 g%), thrombocytopenic and leucopenic, HbF was 23% (1,8 g%) with heterocellular distribution. Globin chain synthesis in the peripheral blood displayed an alpha/non alpha ratio of. 1,05. gamma-Chain analysis was performed: at position 136 the gamma G/gamma A ratio was 3:1 at positions 75 threonine was present in the 30% of the chains. The girl was transfused and oximetholone therapy was started. After 2 months of treatment, an increase of the Hb levels was observed. This improvement lasted about 5 months. The amount of HbF remained constant throughout the management. It may be suggested that androgens work only on HbA synthesis while the HbF-producing-cells are irresponsive to the hormonal stimulation.

Androgens↗
PubMedDate not supplied

[Acute non-lymphatic leukemia in children].

40 children (23 males, 17 females) have been diagnosed have ANLL during the period from february 1970 to september 1981. According to FAB classification, 24 cases were M1,-M2, 9 M3, 3 M4, 3 M5 and 1 M6. At diagnosis, 20 patients (50%) had leukocytes less than 10.000/mmc, 6 (15%) had leukocytes greater than 50.000mmc. Hb levels was 7 g% in 16 patients (40%); 10 children had hepatosplenomegaly (25%), 7 splenomegaly (18%) and 5 lymphoadenomegaly (13%). 4 patients had cutaneous or mucous infiltrates. None had meningeal involvement at diagnosis. According to the year of diagnosis, 3 groups can be identified. In the group I (1970-73), 11 patients have been treated with not codified combination chemotherapy as ARA-C, 6-TG, DNR, CTX, Metil-GAG. In the group II (1974-76) and in the group III (1977-81), the patients (respectively 12 and 17) have been treated according to the following protocols: LAM-5 (3), TRAP (5), COAP (1), LAM 80 (2), AIL 7402 (8), AIL 7604, AIL 7801 (6). Immunotherapy has been performed in 7 cases. CNS prophylaxis (MTX i.t. +/-ARA-C +/- RT) was given in 5 patients of group II and in 6 of group III. I patients of group I (45%), 6 of group II (50%) and 13 of group III (76%) achieved CR. Median duration of remission was 5 months in the group I and in 17 in group II and III.(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis↗
PubMedDate not supplied

[Homozygous beta-thalassaemia and acute lymphoblastic leukaemia (author's transl)].

Two girls with homozygous beta-thalassaemia developed acute lymphoblastic leukaemia. In the first case the two disorders were diagnosed at the same time, when the infant was 11 months old. In the second girl the thalassaemia was diagnosed at the age of 5 months. The patient received monthly packed red cell transfusion. At the age of 3 years she developed the leukaemia. Both the patients achieved a complete remission and are alive after 73 and 5 months from the diagnosis of the leukaemia. The authors discuss the possible relationship between the two haematological diseases.

Child, Preschool↗
PubMedDate not supplied

[Acute lymphoblastic leukemia in adolescents].

Age is an important prognostic factor in acute childhood lymphoblastic leukemia (ALL): intermediate age children (1-9 years) show a better outcome than infants (0-1 year) and adolescents (10-15 years). However recent literature data do not agree about adolescents worse prognosis. We tried to contribute to this issue with a retrospective analysis about presenting features and survival of 302 pediatric patients (65 adolescents and 237 children) with non B ALL enrolled on AIEOP protocols at the Departments of Pediatric Haematology-Oncology (University of Turin) from 1976 to 1992. The last follow up was 30.11.94. We found in adolescents, in spite of higher frequency of unfavourable prognostic factors (Hb > 8 g/dl, mediastinal mass, T cell immunophenotype, L2 morphology), an event free survival similar to children (EFS 52% vs 51%). In conclusion in our population we found that age at diagnosis greater than 10 years does not represent an unfavorable prognostic factor.

Adolescent↗
PubMedDate not supplied

Bone marrow transplantation in Hunter syndrome (mucopolysaccharidosis type II): two-year follow-up of the first Italian patient and review of the literature.

A patient with Hunter syndrome, or mucopolysaccharidosis type II (MPS-osis II), was subjected to bone marrow transplantation (BMT), at the age of 2 9/12 years. A two-year follow-up ensued to the purpose of comparing clinical, biochemical, neuropsychologic status pre- and post-BMT. From the clinical standpoint, a complete normalization of hepatosplenomegaly was observed. In addition the skin decreased in thickness and joint mobility improved. The echocardiography showed normalization of left ventricle size. With the exception of verbal capabilities, there was no further deterioration of the neuropsychologic profile. The ultrastructural examination of the liver showed an almost total disappearance of storage material. Normal iduronate sulfatase levels in leukocytes and lymphoblasts were constantly found after BMT. A qualitative and quantitative improvement in urinary glycosaminoglycan (GAG) excretion was also found. The effectiveness of the BMT in our patient is also assessed in the context of the few cases of MPS-osis II that have been reported to date. A final evaluation of the efficacy of BMT in MPS-osis II will be possible only when a higher number of patients, diagnosed as early as possible and transplanted within the first months of life, can be followed-up for more extended periods of time.

Biopsy, Needle↗
PubMedDate not supplied

Juvenile chronic myelogenous leukemia: report of the Italian Registry. Associazione Italiana di Ematologia Oncologia Pediatrica (AIEOP).

BACKGROUND: Since juvenile chronic myeloid leukemia (JCML) represents no more than 2% of leukemia in children, clinical and investigative experience of this disorder has been limited. In order to evaluate the diagnostic criteria currently applied, to provide centralized facilities for blood culture and to collect data on treatment, and to propose a uniform treatment protocol in our country, a National Registry for JCML was recently established in the "Associazione Italiana di Ematologia Oncologia Pediatrica" (AIEOP). PATIENTS: Out of the 24 cases reported from 9/35 centres, 22 were considered sufficiently documented and were enrolled in the Registry. Clonogenic assay on marrow and peripheral blood cells was performed in all available cases. RESULTS: Common features were non-specific clinical (fever, splenomegaly, hepatomegaly, lymphadenomegaly) and hematologic alterations (anemia, thrombocytopenia, leukocytosis usually < 50 x 10(9)/l, monocytosis, circulating immature granulocytes, increased HbF, normal karyotype). In 11 out of 11 cases, in vitro blood cultures showed the spontaneous growth of CFU-C in the absence of any exogenous source of colony-stimulating activity. Twelve of the 22 patients (55%) are alive (probability of survival 47.7%); most patients were treated according to an acute myeloid leukemia-directed schedule; 5/7 children treated with interferon were alive with disease after a median time of 29 months from diagnosis (range 8-95 months); 4/5 children who underwent bone marrow transplantation were alive in complete remission 10, 24, 42 and 118 months, after the diagnosis. Age < 1 year at presentation was the most significant prognostic factor in terms of probability of survival (80% vs 28%; p = 0.0024). CONCLUSIONS: JCML must be considered in young children for whom acute leukemia has been suspected but ruled out; in vitro cultures should be considered mandatory to confirm the diagnosis. Age less than one year at the presentation was associated with prolonged survival. Only bone marrow transplantation was followed by prolonged disease-free survival, whereas intensive chemotherapy seemed not very effective and potentially associated with life-threatening complications. Interferon therapy appears to be a promising alternative to chemotherapy while the value of unrelated marrow donor is explored.

Adolescent↗