The haemolytic uraemic syndrome of childhood: is living in Belgium a factor of good prognosis?
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Biomedical subjects
Publications and source records attributed to J Ninane.
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A boy, born to a mother with AIDS related complex, was immunised with BCG on the 10th day of life. At the age of 4 months he presented with a local enlarged lymph node, fever, hypotonia, and diarrhoea. Mycobacterium bovis, BCG strain, was grown from the lymph node and cerebrospinal fluid; this proved dissemination.
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To test its diagnostic potential and sensitivity in paediatric malignancy, serum NSE was measured at diagnosis in 191 children with solid tumours and 25 with acute leukaemia. In stages I + II, III + IV and IVs neuroblastoma median levels were 18.0, 91.0 and 24.0 ng ml-1 respectively. For Wilms' patients, median values for stages I, II, III and IV disease were 16.6, 18.0, 29.0 and 47.0 ng ml-1 respectively. High levels of NSE were also found in patients with other types of tumour. Children in clinical remission after treatment for neuroblastoma invariably had normal NSE levels (mean +/- s.d. = 9.2 +/- 3.0 ng ml-1) even though the majority had radiologically identifiable residual disease. The values rose when relapse was radiologically or clinically obvious. We conclude (a) that, though levels of greater than 100 ng ml-1 are highly suggestive of advanced neuroblastoma, caution should be exercised in using serum NSE as a diagnostic test in children with cancer and (b) that serum NSE levels are not a sensitive index of residual neuroblastoma in patients, with initially elevated levels, that are receiving treatment.
Two African infants born in Belgium, dying from the acquired immune deficiency syndrome are reported. The first patient was a premature baby girl born to healthy parents. However, her asymptomatic mother was found to have polyclonal hypergammaglobulinaemia, a reversed T-helper/T-suppressor ratio and a decreased lymphocyte response to mitogens. The second patient's mother was treated for pneumocystis carinii pneumonia during the fourth month of gestation and was subsequently diagnosed with AIDS. The rapid clinical evolution of the acquired immunodeficiency syndrome in infancy suggests that the incubation period is shorter than in adults.
Twenty-five patients with progressive or recurrent neuroblastoma were treated with single-agent ifosfamide. In 2 of 10 relapsing patients, an objective response was observed. If ifosfamide is administered in a dose of 3000 mg/m2 for 2 days every 3 weeks, a response can be expected in 8% of the cases.
An eight-year-old boy with Fanconi's anaemia received a bone marrow transplantation from an unrelated donor. Sustained engraftment was achieved, but he developed major complications including acute grade III graft-versus-host disease, autoimmune haemolytic anaemia with positive Coombs' test and free antibodies against Rhesus group, and a virus-associated haemophagocytic syndrome; this latter complication was successfully treated with acyclovir. Sixteen months after transplantation he has full haematopoietic reconstitution, but with persisting mild autoimmune haemolysis.
Three cases of congenital fibrosarcoma are reported. The use of preoperative chemotherapy, a VAC regimen, allowed conservative surgery in two of them. The three children are well, with no evidence of disease and without sequelae after completion of postoperative chemotherapy.
Typical features of congenital dyserythropoietic anaemia (CDA) were found in a 13-year-old girl admitted for chronic recurrent multifocal osteomyelitis. The findings on light microscopy were in agreement with those described in CDA type II, whereas on electron microscopy, the ultrastructure findings were compatible with both types I and II. The acidified serum lysis test (Ham test) performed with eight normal sera was negative. The patient's red blood cells showed an increased agglutinability with anti-i antibodies. Morphological changes were also shown in the mature neutrophilic granulocyte suggesting that the primary disorder exists already in the multipotent stem cell.
The treatment of neuroblastoma must take into account the prognosis as determined at the time of diagnosis. The stage is an important prognostic factor. In Evans's Stage I neuroblastoma, surgery is curative in itself and adjuvant treatment is not needed. In Evans' Stage II, it is our opinion that the presence of involved lymph nodes worsens the prognosis. Therefore, we suggest that patients with Stage II should be divided into Stage IIA (without lymph node involvement) and Stage IIB (with lymph node involvement). The treatment of Stage IIA patients may be the same as for Stage I patients, i.e., surgery alone. However, Stage IIB patients should be treated with intensive combined chemotherapy of short duration following surgery.
Nine children with poor-prognosis malignancies--seven with advanced neuroblastoma and two with metastatic Ewing's sarcoma--were given high doses of melphalan (HDM), 150 mg/m2 (3 patients) and 180 mg/m2 (6 patients), as a 'late intensification' agent combined with noncryopreserved autologous bone marrow transplants. Melphalan levels in the plasma decreased biphasically, with mean half-lives of 6.6 min and 3.0 h. At the time of marrow reinfusion (12-21 h after HDM) the melphalan plasma level was generally below 0.1 microgram/ml. The renal contribution to melphalan clearance was low, a mean of 5.8% of the injected dose being found in patients' urine over the 12 h following HDM administration. No significant difference was seen in pharmacokinetic parameters between patients undergoing and not undergoing forced diuresis.
We report here on two black African girls who developed an acquired immune deficiency syndrome (AIDS). The first patient was a premature girl born to healthy parents. She suffered from interstitial pneumonitis during the first week of life and died of it at the age of 6 months. Her mother, although asymptomatic, had polyclonal hypergammaglobulinaemia, a reversed T-helper/T-suppressor ratio and a decreased lymphocyte response to mitogens. The second patient had the first symptoms at the age of 6 years, developed a primitive malignant fibrosarcoma of the liver at 8 years old and died 1 year later. AIDS can affect black African children who have not been transfused and whose family members are not considered as at a high risk for this disease. In children, AIDS and cancer can be associated. In the second patient, cytotoxic suppressor lymphocytes (OKT8 positive cells) were shown to behave in vitro as precursors of T-killer cells.
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One dose containing 3,100 PFU of a live attenuated Oka-strain varicella vaccine (Varilrix, Smith Kline-RIT, Belgium) was administered subcutaneously to 45 children, 26 of whom were suffering from acute leukaemia and 19 from solid malignant tumours. Their immunological status had been severely compromised by chemotherapy as evidenced by markedly low values for all immunological parameters. Of the 31 children seronegative for varicella at the time of vaccination, 70%, 85%, 75%, and 40% had varicella serum antibodies at 1, 2, 3, and 12 months after vaccination, respectively. Evaluation of the immunological parameters indicated that they were of no predictive value regarding the antibody response of the patients to the vaccine. Eight children (18%) developed varicella after vaccination. In one case, the disease was caused by the vaccine virus while in another, the vaccine virus was probably also responsible. The remaining six cases were caused by wild virus. The antibody response and accompanying protection against disease induced by the vaccine in severely immunodepressed patients is acceptable, but clearly lower and of shorter duration than in normal subjects. Thus, in immunocompromised patients, booster doses of the vaccine may be required at relatively short intervals.
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