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Biomedical subjects

R F Stevens

Publications and source records attributed to R F Stevens.

At least 73 records · Page 4Linked to original sources
PubMed1988

Nutrition, infection, and morbidity in leukemia.

Seven of the 25 children with acute leukemia were malnourished on admission. Of the 11 children who presented with fever, 6 had protein energy malnutrition and a bacterial infection was confirmed. They had a lower total leucocyte count. Out of 18 well-nourished children, 5 presented with a fever and the causative organism was isolated in 1 patient. There were more patients with a palpable liver in the malnourished group, and they had a high morbidity due to infections during remission induction.

Acute Disease↗
PubMed1987

Mortality and morbidity caused by measles in children with malignant disease attending four major treatment centres: a retrospective review.

Measles is a major cause of mortality and morbidity in children receiving treatment for leukaemia. A review was made of all the documented cases of measles in children in first remission from acute lymphoblastic leukaemia at four major treatment centres in 1974-84. Over the 11 years reviewed 1043 children with acute lymphoblastic leukaemia were referred to these centres. Fifty one (4.9%) died while in first remission and 15 (29.4%) of these deaths were due to measles or its complications: 12 cases of pneumonia, 10 of them fatal; and six cases of encephalitis, five of them fatal and the sixth child left severely handicapped. These children would have had at least a 50% chance of long term survival. The severity of measles in the immunocompromised patient reinforces the need to improve the poor uptake of measles immunisation in Britain.

Acute Disease↗
PubMed1987

Bone marrow transplantation in the treatment of alpha-mannosidosis.

Bone marrow transplantation was performed in a patient with alpha-mannosidosis. To our knowledge this is the first time such treatment has been attempted. The patient died 18 weeks after successful grafting and specimens of tissues were obtained at necropsy. Alpha-mannosidase activity in spleen and liver was just below normal (spleen 102 mumol/g/hour, control 113-330; liver 29 mumol/g/hour, control 30-131). Splenic alpha-mannosidase activity was indistinguishable from the control enzyme with respect to the Michaelis constant, heat stability, and inhibition by cobalt ions, as was 86% of the liver enzyme. In brain tissue alpha-mannosidase activity was 7% of controls, and less than one third had the properties of the normal enzyme. Oligosaccharides were present only in small amounts in liver and spleen, whereas they were greatly increased in brain tissue. Electron microscopic pictures of liver and spleen tissue showed normal morphology, but brain tissue showed definite vacuolation. These findings suggest that transplantation reversed the somatic changes of alpha-mannosidosis but did not affect lysosomal storage within brain tissue. It is concluded that marrow transplantation may not be a suitable treatment for alpha-mannosidosis.

Bone Marrow Transplantation↗
PubMed1987

Indicators of malnutrition in leukaemic children.

In 24 children with acute leukaemia a low serum albumin concentration (31 g/l or less) and a median weight:height ratio of less than 0.95 on admission were indicators of severe weight loss.

Adolescent↗
PubMed1986

The clinical significance of blast cell morphology in childhood lymphoblastic leukaemia.

The morphological classification of ALL based on the FAB co-operative group's criteria is capable of identifying 10-15% of children with L2 disease who, given similar treatment, will fare less well than the 85-90% with the L1 variant. The significant features defining L2 morphology are a low cellular nuclear: cytoplasmic ratio and the presence of nucleoli. Children with L2 disease do not have higher leucocyte counts but are older, have "common" ALL less frequently, and more often have well-preserved marrow function at diagnosis. Their poor outlook is manifest not only by their higher relapse rate but also by a higher proportion failing to remit in the first instance. L2 morphology does not necessarily "breed true" and can arise in a small proportion of patients with previous L1 disease at the time of relapse. Other striking morphological features of lymphoblasts, including azurophil granules, vacuoles and "hand mirror" cells, have yet to have their significance defined, though the latter feature may be an unfavourable finding.

Child↗
PubMed1986

French American British (FAB) morphological classification of childhood lymphoblastic leukaemia and its clinical importance.

As part of the Medical Research Council Leukaemia Trial UKALL VIII, 738 unselected children with acute lymphoblastic leukaemia (ALL) had the morphology of their marrow blast cells reviewed by a panel of three haematologists. Ninety four (13%) showed appearances classifiable as type L2 by the French American and British (FAB) cooperative group's criteria, five (0.7%) were typed L3, and the remaining 639 (86%) as L1. Disregarding the patients classified as L3, those with the L2 variant showed an inferior disease free survival to that of the remainder (p less than 0.01), and more of them failed to remit after receiving "standard" remission induction treatment (p less than 0.01). They included an excess of older children (p less than 0.01) with less profound marrow failure at diagnosis, and fewer of them expressed the common ALL antigen (p = 0.05). There was no association between L2 morphology and the diagnostic white cell count, sex, or the presence of a mediastinal mass. These findings confirm earlier reports that FAB L2 ALL is associated with a poor prognosis and that it occurs more commonly in older children. The high remission failure rate is a recent observation and indicates that alternative early treatment may be appropriate for such patients.

Actuarial Analysis↗
PubMed1986

An unusual presentation of non-Hodgkin's lymphoma (NHL) in a child.

A rare case of a primary early T-cell non-Hodgkin's lymphoma (NHL) in the bronchus of a 10 year old boy is presented. Although histological examination of the biopsied material was compatible with the diagnosis of NHL or carcinoma, the use of monoclonal antibodies showed it to be an early T-cell NHL and not carcinoma. Accordingly the child was treated using a chemotherapy protocol of the UKCCG for NHL. The patient responded so well to his treatment regime that within 2 weeks of the initiation of chemotherapy there was re-expansion of the collapsed lobe. Six weeks later bronchoscopy was entirely normal with no evidence of residual tumour and now 15 months after the time of diagnosis he remains very well with normal chest X-ray.

Antibodies, Monoclonal↗
PubMed1985

Serial incidence of bone marrow GM-CFC prior to the development of acute non-lymphoblastic leukaemia in a child treated for non-Hodgkin's lymphoma.

A child treated for NHL developed acute non-lymphoblastic leukaemia 27 months after stopping treatment. Serial in vitro bone marrow studies showed a normal incidence of GM-CFC following treatment. However, GM-CFC incidence dropped at least 15 months prior to the development of leukaemia. This was associated with an asymptomatic neutropenia but no disturbance of bone marrow morphology. It is concluded that sub-clinical disturbances of bone marrow function may play an important part in leukaemogenesis.

Acute Disease↗
PubMed1983

Liver disease in haemophiliacs: an overstated problem?

Successful percutaneous liver biopsy was carried out on 12 multi-transfused haemophiliacs from the Manchester area with persistently abnormal liver function tests. Only one patient showed evidence of chronic active hepatitis with progression to active micronodular cirrhosis although a further four patients showed some evidence of mild chronic active hepatitis. This represents a much lower incidence of severe histological liver damage than many previous reports and implies that liver biopsy in asymptomatic haemophiliacs may not be indicated as a routine procedure, particularly in the absence of proven therapy. Dynamic liver function tests may prove to be a useful indicator of deteriorating liver function in the otherwise asymptomatic haemophiliac.

Adolescent↗
PubMed1983

Haemangioma with thrombocytopenia (Kasabach-Merritt syndrome).

We describe two patients with haemangioma with thrombocytopenia (Kasabach-Merritt syndrome). Both were treated with corticosteroids without notable improvement. The first patient responded satisfactorily to radiotherapy, whereas the second showed a slow spontaneous resolution.

Female↗
PubMed1981

Comparative studies of a new commercial kit for the estimation of vitamin B12 in serum.

A commercial kit method (Technia Diagnostics) for the estimation of serum vitamin B12 claiming certain practical advantages was examined. Analytical and clinical performance were compared with a non-commercial radioisotope B12 method, previously compared to other commercial radioisotope B12 methods. The kit's analytical performance in our hands was satisfactory, although the within-batch precision and recovery of added cyanocobalamin were disappointing. Clinical performance was comparable with the non-commercial B12 method. Establishment of suitable reference ranges as a prerequisite to diagnostic use is apparent.

Adult↗
PubMed1981

Congenital spherocytosis is often not hereditary.

A retrospective study of 50 patients with hereditary spherocytosis (HS) from 41 families seen between 1967 and 1979 at the Royal Manchester Children's Hospital, and Booth Hall Children's Hospital, Manchester, England is described. Nineteen patients (38%) had no family history of spherocytosis and in 12 patients (24%), tests on the family showed no evidence of disease. Various modes of inheritance are discussed in the light of these results. The traditional name for the condition (hereditary spherocytosis) is considered unsuitable as it is clearly inaccurate in many cases. The name congenital spherocytosis would be more appropriate.

Humans↗
PubMed1980

Plasma exchange and human factor VIII concentrate in managing haemophilia A with factor VIII inhibitors.

Plasma exchanges were combined with human factor VIII concentrate therapy in the treatment of major bleeding episodes in five patients with haemophilia A and factor VIII inhibitors. All patients had a good clinical response to combined treatment. Inhibitor levels showed satisfactory falls before rapid secondary increases of inhibitor levels took place. A sixth patient with von Willebrand's disease and a factor VIII clotting activity inhibitor was successfully prepared for operation using plasma exchange. Postoperative haemostasis and healing were normal. In two patients the plasma exchanges were relatively more effective than the administered human factor VIII in reducing the levels of factor VIII inhibitor. Combined plasma exchange and human factor VIII treatment may offer a rapidly effective means of reducing factor VIII inhibitor levels in this group of patients, together with significant saving of costs.

Factor VIII↗
PubMed1977

A sex difference in the platelet count.

A study of the platelet counts of 868 blood donors aged 18-65 showed a higher mean count in women than in men, the difference in the premenopausal age groups being highly significant (P less than 0.001) and of the order of 40-70 x 10(9)/1. The mean counts of women taking a contraceptive pill did not differ significantly from those who were not.

Adolescent↗
PubMedDate not supplied

Pulmonary function in survivors of Wilms' tumor.

The respiratory status of 47 patients surviving childhood Wilms' tumor was studied. The group that had received flank irradiation (which impinges on the lower lung) (n = 17) had a significantly lower mean percent predicted for forced expiratory volume in one second, residual volume, and total lung capacity when compared to those who had received no irradiation (n = 23). Those patients who had received whole-lung irradiation (n = 3) had significantly lower transfer factor for carbon monoxide and gas transfer per unit lung volume when compared to the nonirradiated group (n = 23). There was no significant difference in the prevalence of respiratory symptoms between the three groups. Patients receiving any form of radiotherapy for Wilms' tumor may have abnormalities of pulmonary function and should have pulmonary function tests performed as part of their long-term follow-up.

Adolescent↗
PubMedDate not supplied

Evaluation of the usefulness of open lung biopsies.

The role of open lung biopsy (OLB) in the diagnosis of the etiology of lung infiltrates in children was analyzed for a 10-year period 1979-1989 in a tertiary referral center. A total of 18 children had 19 lung biopsies to ascertain the cause of lung infiltrates. Thirteen of these children (72%) were immunocompromised due to treatment of hematological/solid malignancies and bone marrow transplantation. The clinical diagnosis was bilateral lung infiltrates of unknown etiology in 17 of 18 children. Eight of these children were ventilated for respiratory failure. The biopsy was useful in achieving a histological diagnosis in 18 of 19 samples (diagnostic yield 95%) and an etiological diagnosis in 14 of 19 samples (etiological yield 74%). Therapeutic strategy was altered in 14 of 18 patients based on the biopsy results. Five of 14 patients responded favorably to a change in specific treatment. The time interval from onset of respiratory illness to biopsy was 2-60 days (mean 16 days). Despite the critical state of these children there were few complications associated with the biopsy and no mortality directly related to the procedure. We recommend that OLB be undertaken sooner rather than later in immunocompromised children with bilateral pulmonary infiltrates of unknown etiology.

Adolescent↗