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Biomedical subjects

R F Stevens

Publications and source records attributed to R F Stevens.

At least 55 records · Page 3Linked to original sources
PubMed1992

Endocrine deficit after fractionated total body irradiation.

Endocrine function was assessed in 31 children (17 boys) after fractionated total body irradiation used in the preparative regimen for bone marrow transplantation. Endocrine dysfunction was present in 25 children. Fifteen of 29 had growth hormone insufficiency 0.9-4.9 years after total body irradiation, yet only three of the 15 had received previous cranial irradiation. Five of 30 had thyroid dysfunction: two with a low thyroxine and raised thyroid stimulating hormone (TSH) concentration and three with a raised TSH and normal thyroxine concentration. Thus the incidence of thyroid dysfunction (16%) is much lower than that reported after single fraction total body irradiation (39-59%). In only two children were abnormalities of the hypothalamic-pituitary-adrenal axis demonstrated. The majority of pubertal children assessed (n = 15) showed evidence of gonadal damage. All the pubertal girls (n = 5) had ovarian failure, although there was evidence of recovery of ovarian function in one girl. All seven boys in late puberty showed evidence of damage to the germinal epithelium, and two of three in early puberty had raised follicle stimulating hormone concentrations. Despite the use of a fractionated total body irradiation regimen, endocrine morbidity is substantial and children undergoing such procedures will require long term endocrine review and management.

Adolescent↗
PubMed1992

Pseudo-Gaucher cells.

A case of acute lymphoblastic leukaemia, associated with cells resembling Gaucher cells in the bone marrow, is reported. The patient had no evidence of inherited Gaucher's disease and the ultrastructural appearance of the cells was consistent with pseudo-Gaucher cells described in other haematological diseases. This is the first report of these cells in association with acute lymphoblastic leukaemia.

Bone Marrow↗
PubMed1992

Detection of bone marrow metastases in neuroblastoma using a short term tissue culture technique.

AIMS: To improve detection of neuroblastoma metastases in the bone marrow: morphological evaluation of bone marrow is a routine and important component of the clinical staging of neuroblastoma and it depends on the successful identification of tumour cells which may only be present at extremely low levels. METHODS: Bone marrow mononuclear cells from patients with neuroblastoma were incubated in a simple suspension culture and examined regularly using an inverted objective microscope. In some cases cytospins of the cultured cells were examined further using morphological and immunocytological techniques. RESULTS: In some cultures spheroidal clumps of cells could be seen growing after only a few days. If the marrow was cultured for a longer period these spheroids continued to increase in size, became adherent to the stromal cell layer on the culture flask floor, and put out long characteristic processes (neurites). Morphological and immunocytological examination of cytospins from these cultures confirmed these cells as neuroblasts. CONCLUSIONS: This method has provided the sole evidence of marrow metastases in several newly diagnosed cases of neuroblastoma in which the bone marrow had shown no evidence of tumour using standard morphological and fluorescent immunocytological techniques. Although negative cultures do not preclude the presence of neuroblastoma, this method is a useful adjunct to the standard techniques.

Biopsy↗
PubMed1992

Prognostic factors in juvenile chronic granulocytic leukaemia.

A retrospective analysis of the clinical and haematological characteristics of patients diagnosed as having juvenile chronic granulocytic leukaemia between 1971 and 1986 was carried out. Thirty-three children were identified who were between the ages of 18 weeks and 8.8 years at diagnosis. The disease was more frequent in boys than girls (23:10). The most common presenting symptoms were skin rash (58%) and bleeding manifestations (45%). All patients had some degree of splenomegaly and in 88% this was more than 3 centimetres below the costal margin. Hepatomegaly and lymphadenopathy were also frequent findings. Anaemia was common and leucocytosis an invariable finding with a white cell count above 50 x 10(9) 1-1 in 42%. Monocytosis was found in 78%. Haemoglobin F measurements were available in 31 children and above 10% in 22 (67%). No child had the Philadelphia chromosome or monosomy 7. Thirty children were treated with chemotherapy, with a variable degree of symptomatic improvement. Twenty-nine patients had died with a median survival time of 5 months. The commonest cause of death was complications of bone marrow failure and no child developed acute leukaemia. Presenting characteristics associated with a longer survival period were age less than 6 months (P = 0.02), female sex (P = 0.02), HbF less than 10% (P = 0.0004) and the absence of bleeding manifestations (P = 0.03). We conclude that the prognosis for children aged over 6 months, with a raised HbF level is very poor, and that, in the absence of possible bone marrow transplantation, consideration should be given to novel treatment approaches for these patients.

Antineoplastic Agents↗
PubMed1992

Intensive chemotherapy with or without additional bone marrow transplantation in paediatric AML: progress report on the MRC AML 10 trial. Medical Research Council Working Party on Childhood Leukaemia.

The Medical Research Council's AML 10 Children Trial commenced in 1988. It is a multicentre collaborative study based on 4 courses of intensive chemotherapy with additional allogeneic bone marrow transplantation for children with a matched sibling donor. The remaining children are randomised either to an autologous transplant using unpurged marrow or stopping therapy. To date 156 eligible patients have been entered with a CR rate of 91%. 56% of children are still alive 2 years after trial entry and 57% are in CR 3 years after achieving CR. The treatment regimen is intensive but mortality and morbidity are acceptable. The study will need to accrue patients for a further 2 to 3 years in the hope of defining the role of allogeneic and autologous marrow transplantation.

Acute Disease↗
PubMed1991

Bone-marrow transplantation for congenital erythropoietic porphyria.

Congenital erythropoietic porphyria, a disorder of haem synthesis, is caused by uroporphyrinogen III synthase deficiency in bone-marrow normoblasts. Uroporphyrins and coproporphyrins accumulate and cause oxidative damage to cells exposed to sunlight. Uroporphyrin overproduction was greatly reduced and skin changes reversed in a girl who received a bone-marrow graft from an HLA-identical sibling at 10 years of age. The patient died 11 months after transplantation because of severe progressive pneumonitis and encephalopathy associated with cytomegalovirus infection, but the encouraging response up to 8 months after engraftment indicates a possible benefit of bone-marrow transplantation in the treatment of this rare but usually fatal inherited disease.

Bone Marrow Transplantation↗
PubMed1991

Acute leukaemias.

Explore the source record for details and available documents.

Adolescent↗
PubMed1991

The role of ondansetron in paediatric patients: a review of three studies.

Emesis is a major problem for children receiving cancer chemotherapy. Three open non-comparative studies have been conducted to assess the safety and efficacy of ondansetron in children receiving a variety of chemotherapy regimens. Patients received various dosages of ondansetron, intravenously or intravenously plus orally, 8-hourly, during chemotherapy, followed by oral treatment 8-hourly for 3-5 days following chemotherapy. Complete control of emesis was achieved in 42-87% of children, and complete plus major control (0-2 emetic episodes) was achieved in 65-98% of children during their chemotherapy treatment, which varied from 1-8 days, and in 54-92% of children over the total study period. Ondansetron was effective in children receiving a wide variety of chemotherapy regimens. However, more intensive chemotherapeutic regimens and platinum drugs are associated with lower response rates. Ondansetron was well tolerated and none of the patients experienced extrapyramidal symptoms.

Adolescent↗
PubMed1990

Ceftriaxone vs. azlocillin and netilmicin in the treatment of febrile neutropenic children.

Efficacy of the cephalosporin, ceftriaxone, was compared with that of the combination of the aminoglycoside, netilmicin, and the penicillin, azlocillin, in the treatment of febrile episodes in immunocompromised neutropenic children undergoing chemotherapy for neoplastic disease. During 100 separate febrile episodes, 40 strains of bacteria were isolated from the blood of 34 patients and a further 55 strains from other sites. Nine strains (four of which were staphylococci) to both netilmicin and azlocillin. There was no difference in clinical response between the two therapeutic regimens as assessed 4 and 7 days after treatment began. Ceftriaxone had the considerable practical advantages of once daily dosage without a need for blood monitoring. Ceftriaxone would appear to be effective as initial monotherapy in the treatment of bacterial infections in severely neutropenic children.

Adolescent↗
PubMed1990

Ribavirin response in measles pneumonia.

A 9-year-old boy with Hodgkin's disease developed measles 1 month after completing eight courses of intensive anti-cancer chemotherapy. The atypical nature of the rash and the absence of Koplik's spots indicated a high risk of progression to fatal giant cell pneumonia. Seven days nebulised and intravenous ribavirin therapy produced apparent recovery. Two weeks later the child presented with measles giant cell pneumonia diagnosed on open lung biopsy. Ribavirin therapy was again successful. The role of ribavirin in the treatment of measles in immunocompromised children is discussed.

Child↗
PubMed1989

Haemolytic uraemic syndrome and the Thomsen Friedenreich antigen.

In three children with haemolytic uraemic syndrome (HUS), evidence of red cell polyagglutinability due to Thomsen Friedenreich antigen (T-antigen) exposure was demonstrated. This was suspected after difficulties in ABO typing and was confirmed using specific antisera. Further supportive evidence included elevation of plasma sialic acid, alteration in red cell surface charge and evidence of T-antigen exposure in the renal biopsy specimen of one patient. Although involvement of this antigen in the pathogenesis of HUS has been associated with a high mortality, all three children have made a complete recovery. With early recognition and subsequent avoidance of plasma products, prognosis of this condition may be improved.

Antigens, Tumor-Associated, Carbohydrate↗
PubMed1989

Laboratory diagnosis and clinical significance of rubella in children with cancer.

Virus-specific antibody responses were studied in 12 children with cancer in whom rubella was diagnosed by seroconversion or a rising titre (greater than or equal to fourfold) of haemagglutination inhibiting (HI) antibody. Our results confirmed the difficulties of making a diagnosis of rubella infection in immunocompromised children using criteria for interpreting antibody assays established in immunocompetent patients. Specific IgM antibody persisted for more than 2 months in 7 of 10 children with probable primary rubella, 3 of whom had high concentrations of such antibody 6, 7 and 11 months after the rash. Radial haemolysis and specific IgG1 and IgG3 antibody responses were low in 4, 2, and 4 patients, respectively. One child apparently had a rubella reinfection and, in another, rubella antibody passively acquired from blood transfusions was probably responsible for the HI seroconversion. Nonetheless, the benign clinical course of rubella in immunocompromised children was confirmed.

Antibodies, Viral↗
PubMed1989

Use of cytotoxic drugs with an end-line filter--a study of four drugs commonly administered to paediatric patients.

The absorption of Novantrone (Mitozantrone), Adriamycin (Doxorubicin Hydrochloride), Cerubidin (Daunorubicin) and methotrexate to 0.2-microM endotoxin-retentive end-line filters was studied under simulated infusion conditions. The drugs were injected into a burette containing saline and then infused using an infusion pump set at 100 ml/h or 80 ml/h. Filtrate samples were collected over a total period of 2.5 h and assayed spectrophotometrically. Recovery of all four drugs following filtration was 96% or greater; indicating absence of absorption by the end-line filters used.

Antineoplastic Agents↗
PubMed1989

Reduced leucine catabolism induced by chemotherapy in leukemic children.

Five normal children, four children on chemotherapy, and two children who had completed chemotherapy within a year were studied using 13C-leucine breath test. 13C-Leucine was administered on an empty stomach and a single breath was collected sequentially over 3 h. The cumulative dose of 13C in expired gas was measured and found to be lower in children who had had chemotherapy than in the normal children. These results suggest that chemotherapy may lead to a reduction in leucine catabolism.

Antineoplastic Agents↗
PubMed1988

Blast cell vacuoles in childhood lymphoblastic leukaemia.

As part of a central review of cell morphology in childhood lymphoblastic leukaemia (ALL), marrow smears from entrants to the Medical Research Council trial UKALL VIII, other than those from children with B-ALL, were studied prospectively for the presence or absence of blast cell vacuoles and for any clinical or biological relevance this feature might have. Adequate slides were available from 733 patients (88% of the trial entrants) after five with B ALL were excluded. Vacuolated blast cells (greater than 10%) were present in 204 (28%). The presence of vacuoles was associated with PAS positivity (chi 2 = 27.8; P less than 0.0001), a diagnostic white cell count (WBC) less than 50 x 10(9)/l (chi 2 = 13.1; P less than 0.0001), and the immunophenotype of 'common' ALL (CD10 positive) (chi 2 = 9.1; P less than 0.01). There was no clear association with French-American-British (FAB) type L1 or L2. The 204 patients with vacuoles had a significantly superior disease free survival compared to the remainder (2P = 0.01), a difference which remained significant when the analysis was stratified by FAB type (2P = 0.01), age (2P = 0.02) or sex (2P = 0.02), but which was lost when stratified by WBC (2P = 0.06). These findings provide further evidence that, outside the context of B-ALL, vacuoles are indicative of a relatively benign disease which responds well to therapy. The French-American-British (FAB) classification should be modified to take this into account.

Adolescent↗