A comparison of oral ondansetron syrup or intravenous ondansetron loading dose regimens given in combination with dexamethasone for the prevention of nausea and emesis in pediatric and adolescent patients receiving moderately/highly emetogenic chemotherapy.

This double-blind, parallel-group, multicenter study compared the efficacy and safety of intravenous (i.v.) ondansetron with oral syrup ondansetron plus oral dexamethasone in the prevention of nausea and emesis in pediatric patients receiving moderately/highly emetogenic chemotherapy. On each day of chemotherapy, patients were administered ondansetron 5 mg/m2 i.v. and placebo syrup orally (n = 215) or ondansetron 8 mg syrup orally and placebo i.v. (n = 223) plus dexamethasone 2-4 mg p.o. Ondansetron 4 mg syrup p.o. was administered twice daily for 2 days following the cessation of chemotherapy. Complete or major control of emesis was obtained in 89% patients in the i.v. group and 88% patients in the oral syrup group during the worst day of chemotherapy treatment (90% CI: -6, 4) and in 85% and 82% patients, respectively, during the worst day of the study period (90% CI: -8, 3). Intravenous or oral syrup ondansetron plus dexamethasone was well tolerated and effective in preventing chemotherapy-induced emesis in pediatric patients.

Limp as unusual presentation of Langerhans' cell histiocytosis.

UNLABELLED: 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. CONCLUSION: A limp can be due to lumbar and paravertebral muscular location of Langerhans' cell histiocytosis.

[Antithrombin deficiency and thrombosis in a young child]. / Déficit en antithrombine et thromboses chez le jeune enfant.

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.

Sweet's syndrome with arthritis in an 8-month-old boy.

Sweet's syndrome was diagnosed in a 4-month-old boy. He was successfully treated with systemic corticosteroids. At the age of 8 months, he developed acute arthritis in his right knee. The synovial fluid was analyzed and revealed a very high neutrophil count and neutrophil activation with a detectable level of intraarticular granulocyte-monocyte colony stimulating factor (GM-CSF). Prednisone injection into the knee led to dramatic improvement. No recurrence occurred. Although arthritis and/or arthralgia are common features in adult patients with Sweet's syndrome, this is the first reported case of Sweet's arthritis in a child.

Fetal rhabdomyomatous nephroblastoma: a tumour of good prognosis but resistant to chemotherapy.

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.

Health care workers: vectors of influenza virus? Low vaccination rate among hospital health care workers.

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.

Haematopoietic stem cell transplantation for sickle cell anaemia: the first 50 patients transplanted in Belgium.

Fifty patients affected by sickle cell anaemia underwent transplantation of HLA-identical haematopoietic stem cells (bone marrow, 48; cord blood, 2). Two groups of patients were considered for transplantation. Group 1 included 36 permanent residents of a European country who, retrospectively, met the inclusion criteria accepted at a consensus conference held in Seattle in 1990, wherein children were selected because they already had evidence of a morbid course. Group 2 included 14 patients who were transplanted earlier, had not received more than three blood transfusions and were transplanted because they had decided to return to their country of origin. Kaplan-Meier estimates of overall survival, event-free survival and disease-free survival at 11 years of the whole grafted population are 93, 82 and 85%, respectively. In group 1, overall survival, EFS and DFS were 88, 76 and 80% and in group 2, 100, 93 and 93%, respectively. Clinical manifestations of the disease, as well as disease associated haemolytic anaemia, disappeared in all successfully treated patients. Recovery of spleen function was present in seven out of 10 evaluated patients. Adverse events (death, absence of engraftment, mixed chimerism and relapse) occurred more frequently in group 1 than in group 2 (25% vs 7%, P< 0.001). Acute graft-versus-host disease (GVHD) was present in 20 patients (grade I or II, 19; grade III, 1), chronic GVHD in 10 (limited, 7; extensive, 3). One patient developed an acute myeloid leukaemia. Gonadal dysfunction was present in all patients (six boys and eight girls) transplanted close to or after puberty, although transient in one adolescent girl.

Chronic relapsing thrombotic thrombocytopenic purpura: three case reports and update of the pathogenesis and therapeutic modalities.

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.

Primary and secondary osteosarcoma of the face: a rare childhood malignancy.

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.

Recommendations on the use of colony-stimulating factors in children: conclusions of a European panel.

UNLABELLED: During 1996 and 1997 a panel of European haematologists, oncologists, and neonatologists developed specific paediatric guidelines for the use of colony stimulating factors based on published literature and the clinical experience of these specialists within each of 13 countries. Well established indications for use comprise intervention in patients with life-threatening infection, adjunctive therapy post autologous bone marrow transplantation (BMT), mobilization of peripheral blood progenitor cells for autologous BMT, patients with acquired aplastic anaemia on anti-lymphocyte globulin and cyclosporin regimen, and severe congenital neutropenia. Less clear indications include primary prophylaxis to support dose intensification in children with high risk/advanced malignancies, secondary prophylaxis to prevent neutropenia in patients with a history of severe neutropenia, support therapy in cases of poor marrow function following BMT and for deteriorating marrow function following successful BMT, in neonatal sepsis and non infectious neonatal neutropenia, in drug induced neutropenia and in HIV-positive patients. Treatment is generally well tolerated and granulocyte colony stimulating factor appears better tolerated than granulocyte and macrophage colony stimulating factor. Economically colony stimulating factors have not been shown to induce excessive costs for a given patient. CONCLUSION: In general the adult guidelines are applicable to children but additional considerations (aggressive or very progressive childhood neoplasms, specific indications, neonatal use, congenital disorders) must be taken into account.

Unifocal cervical Castleman disease in two children.

UNLABELLED: 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. CONCLUSION: 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 Mycobacterium avium infection in a child with decreased tumour necrosis factor production.

UNLABELLED: 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.

[Combined hereditary deficiency in factors VII and X revealed by a prolonged partial thromboplastin time]. / Déficit héréditaire combiné en facteurs VII et X révélé par un allongement du temps de Quick.

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.

Systematic analysis of in-patients' circumstances and causes of death: a tool to improve quality of care.

OBJECTIVES: To point out actions improving quality of care from a systematic analysis of causes and circumstances of death of hospital patients. DESIGN: All in-hospital patient deaths during 1993 and 1994. SETTING: Saint Luc University hospital. MAIN OUTCOME MEASURES: Immediate and original causes of death, other diagnoses or procedures, circumstances of death, place, members of hospital staff, family or friends with the patient, mention of "do not resuscitate", of unexpected death, of problems during the process of care and of autopsy performed. RESULTS: The mean age of the patients was 60 years. The proportion of men was 57%. The majority of patients died in their room. No family or friends, or members of hospital staff were observed for 17%. "Do no resuscitate" was mentioned for 38% of the patients and the proportion of unexpected death and autopsy was 15% and 28% respectively. The most frequent causes of death were malignant and cardiovascular diseases. At least one of the following diseases was mentioned on 14% of the forms; complications during care, septicaemia, pulmonary embolism, adverse effects of drugs or pressure ulcer. Mean length of hospital stay was 14.7 days. CONCLUSIONS: A systematic analysis of the causes and circumstances of death reveals the epidemiology of hospital deaths and highlights specific diseases, complications or circumstances that could be changed. This study creates the opportunity for possible further action, such as improvement in prevention of pulmonary embolism and support of the members of staff or the friends and family of the patient at the time of death.

Radiographic skeletal survey and radionuclide bone scan in Langerhans cell histiocytosis of bone.

BACKGROUND: The lack of a consensus in the literature on the imaging strategy in Langerhans cell histiocytosis (LCH) bone lesions in childhood. OBJECTIVE: To evaluate the relative value of radionuclide bone scan (RBS) and radiographic skeletal survey (RSS) in the detection of LCH bone lesions, both in the initial work-up of the disease and during the follow-up period. MATERIALS AND METHODS: Ten children with bone lesions evaluated by means of RSS and RBS in a retrospective study (1984-1993). RESULTS: Fifty radiologically and/or scintigraphically abnormal foci were detected: 27 anomalies in the initial work-up (12 by both RSS and RBS, 8 by RSS only and 7 by RBS only) and 23 additional anomalies during follow-up (10 by both RSS and RBS, 10 by RSS only and 3 by RBS only). RSS+/RBS- lesions (n = 18) are more frequently encountered in the skull (P = 0.038), and more frequently lack radiologic signs of osteoblastic activity (P = 0.020), than RSS+/RBS+ lesions (n = 22). RSS-/ RBS+ abnormalities (n = 10) were most frequently insignificant. CONCLUSION: In the initial work-up both RSS and RBS should be carried out, while in the follow-up only RSS should be performed.

Persistence of fetal hemoglobin production after successful transplantation of cord blood stem cells in a patient with sickle cell anemia.

A girl with sickle cell anemia was treated with cord blood transplantation combined with hematopoietic growth factor. Cord blood cells were collected from a sister with an identical human leukocyte antigen complex who was a carrier of the sickle cell trait (hemoglobin AS). The patient had complete engraftment and no graft-versus-host disease. The persistence of a high level of fetal hemoglobin 6 months after engraftment was noted.

AIDS-resembling disease in a non-HIV-infected African born to an HIV-positive mother.

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.

HIV Infection in Children Born Before and After Immigration to Belgium.

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.