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

Siblings of paediatric cancer patients: a population at risk.

The psychosocial care of children with cancer has received increasing and welcome emphasis in the medical literature. However, the current and future status of siblings of such patients has been an underestimated area of concern. We examine the general importance of sibling relationships and present the literature regarding siblings of paediatric oncology patients. Donors and recipients of bone marrow transplants are considered specifically. Preliminary results of original research, supported by available published studies, suggest important disturbances in the mental health of siblings. Implications for paediatricians and other health professionals as well as prevention and intervention strategies are indicated.

Bone Marrow Transplantation↗

PubMed1987

Improved detection of metastatic Ewing's sarcoma with the use of bone marrow scintigraphy.

Prognosis in Ewing's sarcoma is inversely related to the extent of the disease at the time of presentation. The most common sites of metastases are the lungs and skeleton. Bone marrow metastases may be present but clinically silent. We report the use of Technetium (Tc)-99m bone marrow scintigraphy to detect sites of marrow involvement by metastatic Ewing's sarcoma. This method of evaluation allowed identification of sites of involvement by Ewing's sarcoma that were not available by any other method of evaluation. In several instances, information provided by this method was pivotal in the management of these patients. Based on this small series of patients, bone marrow scintigraphy appears to be a sensitive modality in the detection of metastatic disease in patients with Ewing's sarcoma. Better understanding of the role of bone marrow scanning and its correlation with other diagnostic procedures in Ewing's sarcoma will require further study.

Adolescent↗

PubMed1987

Rat-bite fever as a cause of septic arthritis: a diagnostic dilemma.

Rat-bite fever results from an infection with the organism Streptobacillus moniliformis. Symptomatic patients often present with fever, malaise, cough, maculopapular rash, and occasional arthritis, and usually have a history of rodent exposure. This report describes a patient with rat-bite fever resulting in suppurative arthritis. The patient's diagnosis was made by culture of S moniliformis from his left wrist. The diagnosis was delayed, however, owing to the lack of an exposure history, atypical clinical presentation, and the unusual microbiologic characteristics shown by this organism.

Arthritis, Infectious↗

PubMed1987

Bone marrow transplantation for malignant histiocytosis.

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Bone Marrow Transplantation↗

PubMed1986

Distribution of immunoreactive peptide B in the rat brain.

Peptide B represents one of the most highly conserved sequences in proenkephalin. To investigate the potential presence of this peptide in the mammalian nervous system, an antiserum raised to this peptide was used to measure the distribution and molecular weight forms of immunoreactive Peptide B in the rat brain. Peptide B-immunoreactivity (ir) was found to be most concentrated in the hypothalamus and the striatum, with lower concentrations in the midbrain and medulla-pons. Characterization of Peptide B-ir by gel filtration demonstrated that the major immunoreactive peak in the hypothalamus corresponded to a peptide with the approximate molecular weight of Peptide B. The major immunoreactive peptide exhibited a retention time on HPLC indicative of a peptide slightly more hydrophilic than bovine Peptide B. The results suggest that proenkephalin in rat brain can be processed to peptides related to bovine Peptide B.

Amino Acid Sequence↗

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

Reptilian enkephalins: implications for the evolution of proenkephalin.

To shed light on the evolution of the opioid peptide precursor proenkephalin, we have investigated the profile of immunoreactive enkephalins in reptilian brain using six antisera directed toward different portions of the proenkephalin molecule. Three different orders of reptiles--turtles, alligators, and lizards--were studied; these orders represent lineages diverging more than 250 million years ago. Reptilian brain was found to contain large quantities of Met5-enkephalin (Met5-enk), Leu5-enkephalin (Leu5-enk), and Met5-enk-Arg6-Phe7 (MERF); gel filtration and reverse-phase chromatography of acid extracts prepared from turtle brain indicated that nearly all of the immunoreactivity corresponding to these peptides could be ascribed to the authentic penta- to heptapeptides. Immunoreactive metorphamide, but not amidorphin, was present in all three species. Unlike mammalian brain, reptilian brain does not contain immunoreactive Met5-enk-Arg6-Gly7-Leu8 (MERGL). These results indicate that reptilian proenkephalin, while similar to mammalian enkephalin in containing Met5-enk, Leu5-enk, MERF, and metorphamide, nevertheless differs from mammalian proenkephalin. Reptilian proenkephalin either may contain the MERGL sequence in a form not recognized by the MERGL antibody, or may lack the MERGL sequence entirely.

Alligators and Crocodiles↗

PubMed1986

Toxicity of benzyl alcohol in adult and neonatal mice.

Benzyl alcohol, a bacteriostatic agent found in many parenteral preparations, has been implicated as the agent responsible for precipitating "the gasping syndrome" in premature neonates. To investigate this toxicity, benzyl alcohol was administered intraperitoneally to adult (23-28 g) and neonatal (2-7 g) CD-1 male mice. Gross behavioral changes were monitored. Low doses (less than 800 mg/kg) produced minimal toxic effects within an initial 4-h observation period. At the end of this time, the LD50 was determined to be 1000 mg/kg for both age groups. When mortality in the adult group was observed after 7 d following a single treatment with benzyl alcohol, the LD50 on day 7 was determined to be 650 mg/kg. Rapid absorption and conversion of benzyl alcohol to its primary metabolite, benzaldehyde, occurred within both experimental groups; the plasma levels of each were comparable in both neonatal and mature animals when determined by GC. In an attempt to alter the toxicity of benzyl alcohol, pyrazole and disulfiram were used to inhibit the activities of alcohol dehydrogenase and aldehyde dehydrogenase, respectively. Treatment with pyrazole, before benzyl alcohol exposure, resulted in an increase in benzyl alcohol levels to 203% of control values and a marked increase in toxicity. Although pretreatment with disulfiram led to benzaldehyde levels which were 368% of control values, toxicity was unchanged. These data imply that the acute toxicity of benzyl alcohol, which includes sedation, dyspnea, and loss of motor function, is due to the alcohol itself and not to its metabolite, benzaldehyde.

Aging↗

PubMed1985

The child with cancer: a psychological overview.

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Adaptation, Psychological↗

PubMed1985

Prognostic factors and outcome in bilateral Wilms' tumor.

Twenty-one patients with bilateral Wilms' tumor are reviewed and the details of diagnosis, therapy, and survival presented. All patients had an abdominal mass at the time of diagnosis. Associated findings included hypertension, aniridia, and genitourinary anomalies. Favorable histologic features were found in all simultaneously occurring tumors and in the initial tumor in nonsimultaneous tumors. Eleven of the 18 patients with simultaneously occurring tumors survived for at least 2 years, for an overall 2-year survival rate of 61%, which was similar to the 2-year survival rate of 60% found in a review of 61 other simultaneously occurring bilateral Wilms' tumors reported in the literature since 1971. Two "front-end" factors that affected prognosis were the patient's age and the stage of the most advanced tumor at the time of diagnosis. A significantly better survival was found in children whose tumor was diagnosed before the age of 2 years and in patients who had Stage I or II disease in the most advanced tumor, as compared with those who had Stage III or IV disease. The overall survival rate in this series and in the literature review is much poorer than that reported for bilateral Wilms' tumor in the National Wilm's Tumor Study; some possible reasons for this are given. The authors' current approach to diagnosis and therapy is reviewed.

Actuarial Analysis↗

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↗

PubMed1985

Acute non-lymphocytic leukemia following multimodality therapy for retinoblastoma.

The genetic form of retinoblastoma carries a high risk of secondary malignant neoplasm, apparently not related to the use of chemotherapy. A child with unilateral non-genetic retinoblastoma who had received chemotherapy and radiation therapy and developed acute non-lymphocytic leukemia (ANLL) is reported. The occurrence of ANLL and retinoblastoma has not been previously reported.

Acute Disease↗