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Biomedical subjects

M R Vowels

Publications and source records attributed to M R Vowels.

52 records · Page 3Linked to original sources
PubMed1987

Aplastic anaemia in childhood: prognosis and approach to therapy.

Thirty-four children with aplastic anaemia who presented between 1964 and 1984 are reviewed. Their ages ranged from one to 13 years (median, seven years). Twelve children had constitutional aplasia; nine of 11 children had responded to androgen and corticosteroid therapy. However, by actuarial analysis, only 48% would survive at five years and only 16% after 10 years. Twenty-two children had acquired aplasia; in 16 children this had no obvious cause. By means of the criteria of the International Aplastic Anemia Study Group, patients were categorized into severe and non-severe groups at diagnosis. Severe disease was present at diagnosis in nine of 22 children with acquired aplasia but in no child with constitutional disease. Two patients with severe acquired aplasia showed a transient response to androgen or corticosteroid therapy, whereas five of nine children with acquired aplasia which was not severe showed a sustained response to such therapy. A significant difference in survival times was seen between severe and non-severe groups at five years; two (17%) of nine children with severe aplasia survived, compared with eight (67%) of 12 children with non-severe aplasia. Three children with severe aplasia who were treated with antilymphocyte globulin showed no improvement. Of five children who underwent bone-marrow transplantation for severe or progressive disease, four survived. It is concluded that bone-marrow transplantation, as a matter of urgency, is the treatment of choice for severe acquired aplastic anaemia; it is also recommended for mild acquired or constitutional aplasia when progressive disease is present or where transfusion therapy will be required. Antilymphocyte globulin is an alternative form of treatment for acquired disease. Androgen therapy is effective in the treatment of non-severe acquired and constitutional aplastic anaemia.

Adolescent↗
PubMed1987

Recent advances in bone marrow transplantation.

The major barriers to successful bone marrow transplantation (BMT) are graft-versus-host disease (GVHD), infection, rejection and relapse. The combination of methotrexate and cyclosporin is significantly better than either alone in controlling GVHD. Removal of T cells from donor marrow prior to BMT has also decreased GVHD significantly, but a 5-10% rejection rate occurs and an increased relapse risk is being reported by some centres. Cyclosporin is valuable in the treatment of both acute and chronic GVHD. Interstitial pneumonitis due to cytomegalovirus (CMV) is a major cause of mortality. Protection can be provided with CMV hyperimmune globulin and also by the avoidance of blood donors who are CMV antibody positive. Fractionated total body irradiation is associated with decreased toxicity compared to single dose. There is a 75% 4 year disease-free survival following BMT for acute non-lymphoblastic leukemia in first remission, a 50% survival for acute lymphoblastic leukemia in second remission and an 88% survival for chronic myeloid leukemia in chronic phase. BMT for beta-thalassaemia major in young patients without organ dysfunction cures 80% of patients and identical results are achieved for severe aplastic anaemia when BMT is undertaken prior to blood product transfusion.

Anemia, Aplastic↗
PubMed1986

Bone-marrow transplantation for haematological malignancy in childhood.

Twenty-three children with haematological malignancies and a poor prognosis underwent bone-marrow transplantation. Thirteen children had acute lymphoblastic leukaemia, eight had acute nonlymphoblastic leukaemia, one had chronic myeloid leukaemia and one had malignant histiocytosis. One child was in relapse at the time of transplant and 22 were in first or subsequent remission. Before transplantation all patients received cyclophosphamide (60 mg/kg) on two consecutive days followed by total body irradiation given as a single dose of 10 Gy at 0.18 Gy/min (one patient) or 0.07 Gy/min (three patients), or as a fractionated dose of 10-12 Gy at 0.07-0.1 Gy/min (19 patients). One child with malignant histiocytosis also received two doses of etoposide (5 mg/kg). Methotrexate was given after transplantation to prevent or modify graft-versus-host disease (GVHD). One patient who received a transplant in relapse died early from overwhelming bacterial sepsis. Twenty-two patients engrafted, and of these 11 developed acute GVHD; five developed chronic GVHD; seven developed interstitial pneumonitis, with four deaths; and five relapsed between three and 12 months after transplantation, with three deaths. Fifty-nine per cent (13/22) of patients who received a transplant during remission remain in continuous complete remission and 68% (15/22) have survived for a median of 18 months (range, four to 73 months). Bone-marrow transplantation that is undertaken during remission of disease offers a prolonged disease-free survival in selected childhood malignancies.

Bone Marrow Transplantation↗
PubMed1986

Bone-marrow transplantation for thalassaemia.

An 18-month-old boy with beta-thalassaemia major underwent bone-marrow transplantation with marrow from his 30-month-old brother. The brother was HLA-identical, mixed-lymphocyte culture non-reactive and had thalassaemia minor. The patient was "conditioned" with busulphan and cyclophosphamide before transplantation and received methotrexate to prevent graft-versus-host disease. Immediately after the transplant, complications arose, which included mild graft-versus-host disease, gastrointestinal bleeding and fever. The boy is alive 18 months after transplantation, is leading a normal life, is receiving no therapy and has a normally functioning donor marrow with thalassaemia minor. Bone-marrow transplantation may be considered as alternative therapy in patients with beta-thalassaemia who are young, and who have no organ dysfunction or iron overload. Chronic transfusion and chelation therapy and its problems must be weighed against the 13% risk of mortality and the 73% chance of a normal life that are associated with transplantation. Older patients, who have received multiple blood transfusions, have iron overload or have organ dysfunction, have a low survival rate after transplantation and this therapy is inappropriate for such patients.

Bone Marrow Transplantation↗
PubMed1985

Bone marrow transplantation for malignant histiocytosis in childhood.

This report describes a girl who was diagnosed with malignant histiocytosis at the age of 5 years. The disease was controlled initially with chemotherapy for 3 years and had then recurred with meningeal involvement on three occasions. Four years and 8 months from diagnosis, bone marrow transplantation (BMT) was undertaken from an HLA-identical and mixed lymphocyte culture (MLC) nonreactive brother after conditioning with VP-16-213 5 mg/kg/day X 2, cyclophosphamide 60 mg/kg/day X 2, and total body irradiation 200 rad twice daily to a total dose of 1000 rad delivered at 7 rad/minute. At the time of transplant, the disease was in remission. Currently, more than 48 months after the transplant, the child remains free of disease, with a normally functioning donor marrow and with no evidence of graft versus host disease. This is the first recorded case of BMT in the treatment of malignant histiocytosis. The outcome in this patient in late-stage disease suggests that BMT could be considered early in management as definitive therapy.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1985

Results of a pilot study for the treatment of childhood acute nonlymphoblastic leukemia.

Eighteen children with acute nonlymphoblastic leukemia were entered on a pilot protocol. The drugs used were vincristine, daunorubicin, cytosine arabinoside, and prednisolone for remission induction, high-dose cyclophosphamide together with vincristine and mercaptopurine for consolidation, and cycles of vincristine, prednisolone, mercaptopurine, methotrexate, and daunorubicin for maintenance therapy. Prophylactic central nervous system therapy (cranial radiotherapy 2400 rad and intrathecal methotrexate 10 mg/m2 for five doses) was given once remission had been achieved. Fourteen of the 18 children (78%) achieved complete remission (CR) and 50% of those achieving CR remain in CR for 35+ to 87+ months. Survival for all children ranges from 2 to 88+ months with 50% remaining alive for 36+ to 88+ months. The protocol was well tolerated with minimal side effects. These results together with those of other recently reported studies indicate an improving prognosis for acute nonlymphoblastic leukemia in childhood.

Acute Disease↗
PubMed1983

Homozygous beta-thalassaemia in a part-Aboriginal Australian.

We describe here the first case of classical homozygous beta-thalassaemia in a part-Aboriginal child. The child came from Bourke, New South Wales, and is the product of a consanguineous mating. His great-great-grandfather was a camel driver from Sri Lanka who settled in western New South Wales. From the family studies, we have found that there are now 17 carriers of beta-thalassaemia in the Aboriginal community in northwestern New South Wales, and it is anticipated that more part-Aboriginal children with thalassaemia major will be identified. The presence of numerous carriers of beta-thalassaemia in the Aboriginal population would suggest that care should be taken in the administration of iron for the treatment of anaemias found in Aboriginal children.

Blood Transfusion↗
PubMed1980

Erythrocyte phosphoglycerate kinase deficiency: enzymatic and oxygen binding studies.

Enzyme and oxygen transport studies have been made on seven members of a kindred with red cell deficiency of phosphoglycerate kinase (PGK), an enzyme known to be linked to the X-chromosome. The two affected males were children, had little detectable PGK activity and had severe neurological damage. Both had moderate haemolytic anaemia complicated by the occurrence of haemolytic crises. Red cell ATP levels were substantially decreased in both subjects. However, they had more than double the normal level of 2,3-diphosphoglycerate (2,3-DPG) in their red cells, associated with a rightward shift in their oxygen-haemoglobin equilibrium curves. This shift in the curve was sufficient to permit oxygen delivery to most body tissues, including the brain, at better tensions than normal, except during a haemolytic crisis. Two of the three known heterozygotes were aged over 70. They had moderate haemolytic anaemia, considerable reduction of red cell PGK activity (19% and 35% of normal, respectively), and considerable increase in red cell 2,3-DPG with rightward shifting of the oxygen-haemoglobin equilibrium curve. The other known heterozygote was aged 22 and showed little abnormality in her blood oxygen transport.

Adenosine Triphosphate↗