Biomedical subjects
Publications and source records attributed to J Ninane.
Two years after an orthotopic liver transplantation, a multifocal hepatic tumor with lymphonodular metastases, identified as a fibrosarcoma, developed in a 4-year-old girl being treated with cyclosporine. On a needle biopsy sample, genetic typing of the HLA-DR group revealed that tumoral cells were from the recipient.
Six children with leishmaniasis, aged 10 months to 10 years, were treated in the Paediatric Department. Four patients had visceral leishmaniasis (kala-azar): diagnosis was based on bone marrow examination and therapy consisted of a combination of Glucantime and Lomidine. The remaining two children had cutaneous leishmaniasis: diagnosis was made by skin biopsy and the patients were treated with Glucantime alone. In all children, serology was clearly positive at the time of the diagnosis and all patients improved. The only side effects were cough associated with fever in one child, and supraventricular premature beats in another one. They were ascribed to Glucantime, and proved reversible after discontinuation of the treatment.
We report our experience of renal transplantation in three patients treated for Wilms' tumor (with lung metastasis in two of them), and review 26 previously reported cases in order to define the current indications of transplantation in this setting. Our patients, aged 5-12 years, were transplanted 13-95 months after completion of Wilms' tumor treatment. All three are alive and tumor-free, two with a functioning graft 20 and 97 months after transplantation. Two findings emerge from the review of the literature. First, posttransplant mortality is influenced by the delay between completion of tumor treatment and transplantation. Mortality reaches 79% when that delay is less than one year but falls to 27% when that delay exceeds one year. Second, the prognostic value of pretransplant metastasis depends on its location. All four patients with pretransplant abdominal metastasis died with active metastatic disease. By contrast, of three patients treated before transplantation for metastasis confined to the lung, two are alive and tumor free. We conclude that renal transplantation should be offered to patients successfully treated for Wilms' tumor for at least one year, even if the disease has been complicated by pulmonary metastasis. Several long-term survivors attest that the disease can be cured even under maintenance immunosuppression.
Sixteen children, aged 16 days to 13 years with hepatoblastoma (HB) (13 patients) or hepatocellular carcinoma (HCC) (3 patients), were given a total of 89 courses of cisplatin and doxorubicin (PLADO) as IV continuous infusion. All tumors were confined to the liver except for 1 hepatoblastoma patient with pulmonary metastases at presentation. Tumor response to PLADO was evaluable in 10 children (8 HB, 2 HCC) treated with preoperative chemotherapy and in another 2 HB patients treated when they developed pulmonary metastases after initial treatment with surgery alone. There were 2 complete responses (2 HB with pulmonary recurrences), 7 very good partial responses (6 HB and 1 HCC), 2 partial responses (1 HB, 1 HCC), and 1 stable disease (HB). The last patient underwent orthotopic liver transplantation whereas all the other patients had their tumor completely excised at delayed surgery. Documented toxicity was BM depression (16 patients), infection (11), vomiting (11), mucositis (3), hearing loss (1), and cardiotoxicity (1). These data indicate that PLADO in continuous infusion is effective in the treatment of malignant epithelial liver tumors with acceptable toxicity.
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Sickle cell anaemia is still responsible for severe crippling and death in young patients living in developing countries. Apart from prophylaxis and treatment of infections, no active treatment can be safely proposed in such areas of the world. Therefore a bone marrow transplantation was performed in 12 patients staying in Belgium and planning to return to Africa. Twelve patients, aged between 11 months and 23 years (median 4 years), underwent a HLA identical bone marrow transplantation. The conditioning regimen included oral busulphan for four consecutive days (4 mg/kg) followed by four days of intravenous cyclophosphamide (50 mg/kg). In 10 patients the engraftment was rapid and sustained. A further patient suffered transient red cell hypoplasia and another underwent a second bone marrow transplantation from the same donor at day 62 because of graft rejection. All patients are alive and well with a follow up ranging from 9-51 months (median 27 months). In all cases a complete cessation of vaso-occlusive episodes and haemolysis was observed as was a change in the haemoglobin pattern in accordance with the donor's electrophoretic pattern.
The authors report a case of reversal of urinary diversion in a 14 year old boy who had presented, three years previously with an embryonal rhabdomyosarcoma of the base of the bladder. The initial treatment had consisted of preoperative and postoperative chemotherapy combined with radical cystoprostatectomy with urinary diversion via a colonic conduit. The principle of the technique of reversal of urinary diversion are discussed in the light of this unusual case.
We report the case of a family in which the mother died of hypertensive encephalopathy following the relapse of a phaeochromocytoma. Two of her children are still alive. Both children had malignant phaeochromocytomas that have been treated by surgery and 131I-MIBG. The first child presented with phaeochromocytoma of the right suprarenal gland at the age of 7 years. Surgery was performed. At the age of 14 years, he developed a tumour of the left suprarenal gland and two pulmonary metastases demonstrated by 131I-MIBG. The three tumours were removed, but new lesions occurred. The boy then was treated with 200 mCi (7,400 MBq) of 131I-MIBG given twice, and is now free of disease more than 2 years after treatment. His sister presented at the age of 12 years with phaeochromocytoma of the left suprarenal gland, the only lesion recognized by 131I-MIBG. The tumour was removed, but 5 months later, she developed phaeochromocytoma in the right suprarenal gland. She was treated with 200 mCi (7,400 MBq) of 131I-MIBG and surgery was performed 6 months later. Histology of the suprarenal gland could not demonstrate the persistence of phaeochromocytoma cells. The child is now free of disease more than 2 years after treatment.
Out of our series of 24 children seropositive for the Human Immunodeficiency Virus (HIV), parotid gland enlargement was noted in 4 children with AIDS-related complex (ARC) presenting also a Lymphocytic Interstitial Pneumonitis (LIP) on their chest radiographs. The ultrasound (US) aspect of the parotid gland suggests acinar enlargement (suggesting the presence of lymphocytic infiltration). The aspect displayed in the parotid mirrors the process developing in other areas (lungs, liver, spleen, lymph-nodes), i.e. a syndrome of lymphocytic (CD8) proliferation present at the stage of ARC.
A case of infantile myofibromatosis associated with oesophageal atresia, annular pancreas, additional sacral vertebra and hypoplatic right kidney in a male neonate is reported. The possibility of associated malformations in this rare disease is outlined.
Between 1969 and 1988, 146 children with immune thrombocytopaenic purpura (ITP) were seen in the outpatient clinic. The diagnosis was based upon an isolated thrombocytopaenia, without disseminated intravascular coagulation, splenomegaly or systemic disease and a normal bone marrow. Patients who required treatment (116/146) received either steroids (105/116) or IV gammaglobulins (IV-Ig) (11/116) as initial therapy. The long term outcome was similar in both groups. IV-Ig had the advantage to give a rapid increase in the platelet count without major side effects and to be very useful in chronic ITP either as maintenance therapy or as preparation for splenectomy.
Juvenile laryngeal papillomatosis is a rare and benign tumoral disease of childhood characterized by numerous relapses despite complete resection. The ENT treatment of choice is to vaporize the papillomas with a CO2 laser. Since the discovery of a viral etiology (Human Papilloma Virus), resection has been followed by medical attempts to control the disease by using various antiviral treatments. Among the latter, alfa interferon has proved effective during the first six months of treatment. In this article, we report on five cases of refractory juvenile laryngeal papillomatosis treated by excision (CO2 laser in four children, surgical resections in one child) and alpha-r IFN 1.5 x 10(5) U/kg daily. With this strategy, three of the five children are currently disease-free for periods ranging from 22 to 68 months. This series includes one remarkable observation of one child who responded only to double doses of alpha-r IFN, after initial failure at conventional doses. This therapeutic scheme reduced the frequency of relapses in a fourth child. In only one child the treatment did fail to modify the natural course of the disease. Side effects were tolerable and included anorexia (one case), palmar erythema (one case), a flu-like syndrome (two cases) and mild transient transaminase rise (three cases) not precluding further treatment. CO2-laser caused one laryngeal oedema and synechia of the anterior commisure of the vocal laryngeal cords in one other case.
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We report three siblings (2 F, 1 M) aged 9, 12, and 15 years with a thrombocytosis, above 650 X 10(9)/l for at least 18 months and a leukocyte alkaline phosphatase score below 37. The diagnosis of essential thrombocythemia was based upon the exclusion of a reactive thrombocytosis or another myeloproliferative disease. Cytogenetic examination of marrow cells was normal.
Five children with aplastic anemia (AA) were treated with Cyclosporine A (CyA) after failure or poor response to other immunosuppressive agents. A complete remission was achieved in 3 patients, a good partial response in 1, and a poor partial response in another. In 1 patient in whom a complete remission was obtained, a relapse occurred 4 months after CyA was stopped. The treatment was resumed and a complete remission was again observed. Four patients were still on treatment, and side effects were mild. CyA appeared to be an effective treatment in AA.
The pharmacokinetics of alkylating activity were studied in 17 children treated i.v. with ifosfamide (IF) at 3 g/m2 as a 1-h infusion for 2 consecutive days every 3 weeks, with mesna as a uroprotector. Two patients were treated for a newly diagnosed rhabdomyosarcoma according to the current SIOP (International Society of Pediatric Oncology) protocol. The other 15 patients were treated in a phase II study and presented with one of the following malignancies in relapse: neuroblastoma (7), osteosarcoma (3), soft tissue sarcoma (2), Wilms' tumor (1), non-Hodgkin's lymphoma (1), and acute lymphoblastic leukemia (1). Plasma alkylating activity levels determined by using 4(4'-nitro-benzyl)-pyridine showed considerable inter-individual and intercyclic variations and decreased biphasically, with mean alpha and beta half-lives of 60 min and 6-7 h, respectively. Probably as a result of liver mixed-function oxidase induction, on the 2nd day of treatment the terminal half-lives were shorter, the plasma exposures were lower, and the mean plasma clearances were higher. Renal excretion was almost complete after 24 h, accounting for a mean of 19% of the injected dose. The CSF alkylating activity levels, obtained in four children, were always lower than the plasma levels and ranged from 8 to 51 micrograms/ml, with a mean CSF/plasma ratio of 0.53 +/- 0.23 during the first 12 h. We conclude that IF alkylating activity was biphasically cleared from the plasma, with significant interindividual and intercyclic variability, that the renal contribution to the clearance was low, and that high levels of CSF alkylating activity could possibly contribute to the CNS toxic side effects observed in pediatric patients treated with high-dose IF/mesna.
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The case of an 11-year-old boy suffering digital fibrous tumor of childhood with multiple recurrences is presented. The possible benefit of chemotherapy instead of a high-level amputation is discussed.