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Oral candidosis in neonates and children is a common infection which occurs often during the first few months after birth, but occasionally also in older children with certain predisposing factors. In neonates, oral candidosis is usually benign, although the symptoms of such an acute infection can be-disturbing to both the patient and the parents. In older children developing oral candidosis, specific predisposing factors may be present (e.g. immunodeficiency, chemotherapy, etc.). In such cases, the infection may constitute a source for further dissemination, leading to occasionally fatal Candida sepsis or to widespread chronic mucocutaneous candidosis. Treatment modalities to date include drugs with limited or no absorption from the gastrointestinal tract (e.g. nystatin and miconazole) and agents that are absorbed, combining local effect with systemic therapy (e.g. clotrimazole, ketoconazole, itraconazole and fluconazole). Overall, it appears that treatment of neonatal oral candidosis should be performed with non-absorbable drugs, while the systemically active agents should be used primarily if a risk of dissemination exists or if widespread disease is present. In general, side-effects and toxicity are not major causes of concern with non-absorbed or absorbed antifungals in children with oral candidosis, since treatment is usually of relatively short duration. When the systemically active agents are used in premature infants with suboptimal liver function, the risk of drug-induced liver toxicity may be increased.

Fetal rhabdomyomatous nephroblastoma (FRN) is a rare variant of Wilms' tumour. The tumour chiefly consists of fetal striated muscle with particularly distinct striations and central nuclei. To determine the effect of (preoperative) chemotherapy in the treatment of this subtype of nephroblastoma, a retrospective analysis was performed. By 1 November 1991, SIOP 9 had registered 852 patients (pts) from 55 centres. We report on 13 children diagnosed with FRN between 1988 and 1992 with a median age of 2 years and 1 month (range 1 month-8 years 6 months). There were 7 boys and 6 girls. 9 patients were classified as stage I, 2 as stage II, 1 as stage III and 1 as stage V. 12 patients received preoperative chemotheraphy with actinomycin-D and vincristine for 2 weeks (1 pt), 4 weeks (5 pts) and 8 weeks (6 pts) respectively. The average tumour volume at registration (determined by ultrasonography) in 12/13 patients was 965 cm3 (range 17.3-2520 cm3). 3/7 of the FRN patients showed no tumour regression after 4 weeks preoperative CT and 4/8 after 8 weeks preoperative chemotheraphy (compared with only 28 and 34%, after 4 and 8 weeks CT, of all trial patients). Of 13 patients, 10% are alive and free of disease with a mean follow up of 4 years. This variant of Wilms' tumour is a poor responder to preoperative chemotherapy and is associated with a generally favourable outcome in most of all unilateral cases when treated by surgery.

Chronic relapsing thrombotic thrombocytopenic purpura (CRTTP) is the rarest type of TTP, usually presenting in childhood. The aetiology is still not fully explained. The disorder is associated with the presence in plasma of unusually large von Willebrand factor (UlvWF) multimers that are especially prominent between episodes. CRTTP can be prevented by periodic plasma transfusions. We report three children with congenital CRTTP who have been successfully treated and maintained in prolonged remission by the prophylactic use of fresh frozen plasma without concurrent plasmapheresis. Two of the patients are sibs.

BACKGROUND: Osteosarcoma of the head and neck, especially primary forms, remains a rare and highly malignant tumor. PATIENTS: This report describes two patients who developed an osteosarcoma of the face more than ten years after treatment for bilateral retinoblastoma. We also report a third patient who presented with a primary osteosarcoma of the right superior maxilla which is one of the rarest tumors encountered in childhood oncology. CONCLUSIONS: The mainstay of therapy is surgical resection with negative margins. Careful, long-term follow-up of survivors of hereditary retinoblastoma is essential, especially for those given radiation therapy.

Background - Congenital factors VII and X deficiency is rare. Association of both deficiencies is exceptional. Case report. - A 3 year-old boy, born to consanguinous Moroccan parents, had a prolonged partial thromboplastin time discovered fortuitously. This finding led to the diagnosis of combined factors VII and X deficiency. His siblings had the same deficiencies. Conclusion. - Profound deficiencies in factors VII and X are inherited following an autosomal-recessive mode. These deficiencies may be asymptomatic, only discovered by prolonged partial thromboplastin time. They may also be revealed by intracranial bleeding and other severe hemorrhages. Treatment consists of administration of factor VII or PPSB.

Methods: The records of 119 children, either infected with HIV or born to HIV-infected mothers were reviewed. This group was mainly represented by children whose families came to Belgium from Central Africa. Seven children were infected through blood or blood products transfusion, whereas the remaining 112 were born to infected mothers. Most of the latter were infected through heterosexual contacts. Results: The overall rate of perinatal transmission was 44%. The transmission rate observed in our population is higher than in other European studies and closer to African studies. Lymphadenopathy was observed in 86% of infected children versus 16% in noninfected children during the first year of life (p =.0002). Serum IgG level was higher in infected children than in noninfected children. Conclusions: HIV transmission is firmly related to breast feeding.

We report the case of a 10-month-old boy born to an HIV-positive mother who presented with symptoms compatible with AIDS. However, he became HIV-negative and his condition was probably due to combined cytomegalovirus and Epstein-Barr virus infections.

We report the case of a young girl who had hemihypertrophy and developed Wilms' tumor in both kidneys and clear-cell adenocarcinoma of the uterine cervix with no maternal history of stilbestrol ingestion during pregnancy. Karyotype on the second Wilms' tumor showed monosomy 22. The link between hemihypertrophy and Wilms' tumor is well known, but their association with clear-cell carcinoma of the cervix has not been previously described. (C) 1993 Wiley-Liss, Inc.

Cellular (or atypical) congenital mesoblastic nephroma (CMN) is a potentially aggressive form of the benign classical congenital mesoblastic nephroma. We report here a case of cellular CMN in a 5-month-old boy treated preoperatively with chemotherapy with an excellent response allowing a complete surgical resection.

Between 1985 and 1989, eight children underwent two successive bone marrow transplantations. The initial disease was chronic myelomonocytic leukemia in three patients, chronic myelocytic leukemia in two, acute M7 nonlymphoblastic leukemia in one, sickle cell anemia in one, and thalassemia major in one. The preparation in view of the second grafting included high-dose chemotherapy in all patients, associated with antithymocytic globulin transfusion and total nodal irradiation in three patients. Hematological recovery was similar after both graftings. Infectious complications were not more common following the second graft than after the first one. On the other hand, the rates of rejection and graft-versus-host disease were lower, probably due to a more intensive immunosuppressive therapy. The prognosis of chronic leukemia relapsing after a first graft does not seem to be improved by a second attempt.

An isolated eosinophilic granuloma involving the posterior elements of a lumbar vertebra is reported in a 3-year-old boy presenting with progressive limp. Radiological investigations revealed osteolysis of the L5 right pedicle. MRI showed a well-defined homogeneous mass with nonspecific signal intensity. An unusual feature was the paravertebral muscular location of the largest part of the tumour indicating a possible soft tissue origin. Immunohistochemical studies were typical for Langerhans' cell histiocytosis.A limp can be due to lumbar and paravertebral muscular location of Langerhans' cell histiocytosis.

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.

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.

Background. - Thromboses represent a rare event in children and may be due to a deficiency of antithrombin. Case report. - A 10-year-old boy developed thrombosis due to a congenital quantitative deficiency in antithrombin, confirmed by molecular biology. His father was diagnosed with the same deficiency. The child was first treated with heparin and is now on antivitamin K. He is well 26 months after diagnosis. Conclusion. - When a young patient presents with a thrombotic event, a congenital deficiency in one of the inhibitors of coagulation, one of which is antithrombin, should be looked for and the condition treated as soon as possible. (C) 1999 Elsevier, Paris.

The influenza vaccination rate among health care workers is not very high and needs to be improved. The authorities of our university hospital decided to encourage vaccination among health care workers and to include it in the hospital quality of care program. A survey about knowledge of influenza disease and its vaccination was undertaken, and 37.2% of the workers participated in the survey. Among them, 31.8% were vaccinated. The vaccination rate was higher when workers were older, men, physicians, technicians, or laboratory workers, vaccinated in the previous flu season and had fewer contacts with patients. The variables with the highest impact on the vaccination rate were vaccination the previous year, belief that vaccination is highly effective, and affirmation that prevention is important. Our study shows that an active vaccination campaign is also necessary for health workers to put them in the stream of usually vaccinated people.

Castleman disease (CD) is a benign lymphoproliferative disorder characterized by enlarged hyperplastic lymph nodes. Most observations focus on adult patients. In children the disease is rare, usually localized and may be symptomatic or asymptomatic. We reported two children, 7 and 14 years old respectively, affected by unifocal cervical CD of the hyaline vascular type.Two cases illustrate that CD may mimic a neoplasm and should be considered in presence of a solitary neck mass with specific features on CT and MRI.

Disseminated atypical mycobacterium infection is essentially reported in cellular immunodeficient children. Cell-mediated immunity including cytokines like tumour necrosis factor alpha (TNF) and gamma interferon (IFN) is the most important factor allowing control of the dissemination of Mycobacterium. We report a child with disseminated Mycobacterium avium infection without classical immunodeficiency or HIV infection. Immunological studies revealed a defect of TNF production when the monocytes of the patient were primed with endotoxin (Escherichia coli). CONCLUSION: This patient represents a further case of possible macrophage defect explaining the susceptibility to intracellular pathogens.

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.

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.

We report the occurrence of a leiomyoma of the suprarenal gland in a 10-year-old girl with ataxia-telangiectasia (A-T). Muscle cell tumors are very uncommon in this gland as they are in A-T. Possible reasons for developing nonhematologic tumors in this syndrome are reviewed. A defect in DNA repair mechanisms probably favors, in young children, the expression of tumors normally expected in the aged.

A 9-year-old boy presented with a small subcutaneous tumor of the trunk and diffuse bone marrow involvement. The first histological diagnosis given was undifferentiated malignancy possibly of neural crest origin and chemotherapy was started immediately using vincristine, cyclophosphamide, cisplatin, and teniposide (OPEC). Complete response was achieved after four courses of chemotherapy. Histological slides were then reviewed and the final diagnosis of alveolar rhabdomyosarcoma (RMS) was retained. Moreover, chromosome analysis of malignant cells in the bone marrow revealed a translocation involving chromosomes 2 and 13:t(2;13) (q35;q14). This specific karyotype finding has been recently reported in a few cases and could be specific for alveolar RMS. The patient had a relapse 7 months after diagnosis and died 4 months later.

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), vomitting (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.

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.

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.

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.

Two consecutive nonrandomized studies were conducted in children with prolonged granulocytopenia to evaluate the prophylactic antifungal activity of ketoconazole and the new triazole itraconazole. The conditions were equivalent in both studies. The incidence of colonization was 10% in the ketoconazole group and 19% in the itraconazole group (this difference is not significant). For suspected and proven infections, the incidence was 5% for ketoconazole and 10% for itraconazole, but the incidences were too low for statistical comparison. Although the colonization rate was higher for itraconazole, there was no sign of Aspergillus, whereas in the ketoconazole group an autopsy, proven aspergillosis has been reported.

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.

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.

Five children with severe sickle cell anaemia underwent an HLA compatible allogeneic bone marrow transplantation. In four children the engraftment was rapid and sustained. The fifth child rejected the bone marrow graft and required a second bone marrow transplantation 62 days after the first one. The outcome was then uneventful. In all cases there was complete cessation of vaso-occlusive episodes and haemolysis. The haemoglobin electrophoretic pattern became similar to that of the donor (AA or AS) and cytogenetic studies in three patients confirmed the donor origin of bone marrow cells.

Humoral factors influencing granulopoiesis have been evaluated using diffusion chambers (DC) implanted in the peritoneal cavity of mice challenged by an aseptic abscess produced by the subcutaneous implantation of copper rods. This resulted in an increase in peripheral blood neutrophils and an increase in tibial granulocytic elements. When DC loaded with bone-marrow cells were implanted into mice stimulated the day before by an aseptic abscess significantly more CFU-s, CFU-c, proliferative and non-proliferative granulocytes were produced, as compared to DC implanted into control hosts. When DC were implanted 4-6 d after the induction of inflammation in mice a significant depression of DC granulopoiesis was observed. Levels of serum and DC fluid CSF and serum inhibitors of in vitro colony growth showed no correlation with DC myelopoiesis. The data show that mice undergoing an inflammatory reaction elaborate first humoral substance(s) enhancing CFU-s and granulocytic growth in DC and next inhibitory factor(s) of DC granulopoiesis.

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 studied the effect of lithium on diffusion chamber (DC) granulopoiesis. When DC loaded with bone marrow cells were implanted into the peritoneal cavity of mice previously injected with lithium carbonate, more proliferative and nonproliferative granulocytes were produced as compared to DC implanted into control hosts. The number of DC CFU-c was increased significantly in the lithium-treated group, but there was no difference in the number of DC CFU-s. Levels of DC fluid CSF showed no evident correlation with DC myelopoiesis. These data suggest that a humoral factor other than CSF mediates the action of lithium in DC granulopoiesis, and that lithium's influence on DC hematopoietic stem cell proliferation occurs mainly at the CFU-c level.

Cytogenetic examination of bone marrow cells from two patients with juvenile chronic myelogenous leukemia (JCML) showed a monosomy 7. Peripheral blood from these two children was found to contain large numbers of cells capable of erythroid colony formation in tissue culture without added erythropoietin. We conclude that erythroid progenitor cells are part of the malignant clone in JCML.

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.

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.

Leukaemic infiltration of the anterior chamber of the eye was the only sign of relapse in two children with acute lymphoblastic leukaemia who had been off treatment for two and four months. The malignant cells had probably been present in the eye since the onset of the disease.

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.

HIV infection has a wide spectrum of clinical expression in children ranging from no symptoms to full-blown AIDS. The case definition for pediatric AIDS provided by the US Centers for Disease Control has been modified twice. Each modification has included more diseases indicative of AIDS in children and more children are being reported with a broader spectrum of manifestations. In this study the 3 successive case definitions were applied to 47 children of African origin with a symptomatic HIV infection. Of these children 19% met the 1st CDC case definition 23% met the 2nd and 72% met the 3rd; thus 28% of these chidren did not fulfil any of the CDC definitions. The features esponsible for this increase were presumptively diagnosed interstitial pneumonia repeated bacterial infections presumptively diagnosed Pneumocystis carinii pneumonia HIV encephalopathy and/or generalized cytomegalovirus infection. Only 4 of these 23 children have died all before the age of 1 year. In summary the 3rd CDC case definition has allowed physicians to report most of the severely affected children with HIV infection but it also included children with a more chronic pattern of disease and a better prognosis.