Combined therapy in children and adolescents with classical Hodgkin's lymphoma: A report from the SFCE on MDH-03 national guidelines.

Hodgkin's lymphoma (HL) in children and adolescents is highly curable, but children are at risk of long-term toxicity. The MDH-03 guidelines were established in order to decrease the burden of treatment in good-responder patients, and this report should be considered a step toward further optimization of treatment within large collaborative trials. We report the therapy and long-term outcomes of 417 children and adolescents treated according to the national guidelines, which were applied between 2003 and 2007 in France. The patients were stratified into three groups according to disease extension. Chemotherapy consisted of four cycles of VBVP (vinblastine, bleomycin, VP16, prednisone) in localized stages (G1/95 pts/23%), four cycles of COPP/ABV (cyclophosphamide, vincristine, procarbazine, prednisone, adriamycin, bleomycin, vinblastine) cycles in intermediate stages (G2/184 pts/44%) and three cycles of OPPA (vincristine, procarbazine, prednisone, adriamycin) plus three cycles of COPP in advanced stages (G3/138 pts/33%). Radiation therapy of the involved field was given to 97% of the patients, with the dose limited to 20 Gy in good responders (88%). With a median follow-up of 6.6 years, the 5-year event-free survival (EFS) and overall survival (OS) were 86.7% (83.1-89.7%) and 97% (94.5-98.1%), respectively. EFS and OS for G1, G2, and G3 were 98% and 100%, 81% and 97%, and 87% and 95%, respectively. Low-risk patients treated without alkylating agents and anthracycline had excellent outcomes and a low expected incidence of late effects. Intensification with a third OPPA cycle in high-risk group patients, including stage IV patients, allowed for very good outcomes, without increased toxicity.

Childhood acute lymphoblastic leukaemia and indicators of early immune stimulation: the Estelle study (SFCE).

BACKGROUND: Factors related to early stimulation of the immune system (breastfeeding, proxies for exposure to infectious agents, normal delivery, and exposure to animals in early life) have been suggested to decrease the risk of childhood acute lymphoblastic leukaemia (ALL). METHODS: The national registry-based case-control study, ESTELLE, was carried out in France in 2010-2011. Population controls were frequency matched with cases on age and gender. The participation rates were 93% for cases and 86% for controls. Data were obtained from structured telephone questionnaires administered to mothers. Odds ratios (OR) were estimated using unconditional regression models adjusted for age, gender, and potential confounders. RESULTS: In all, 617 ALL and 1225 controls aged ⩾1 year were included. Inverse associations between ALL and early common infections (OR=0.8, 95% confidence interval (CI): 0.6, 1.0), non-first born (⩾3 vs 1; OR=0.7, 95% CI: 0.5, 1.0), attendance of a day-care centre before age 1 year (OR=0.7, 95% CI: 0.5, 1.0), breastfeeding (OR=0.8, 95% CI: 0.7, 1.0), and regular contact with pets (OR=0.8, 95% CI: 0.7, 1.0) in infancy were observed. CONCLUSIONS: The results support the hypothesis that conditions promoting the maturation of the immune system in infancy have a protective role with respect to ALL.

Acute childhood leukaemia and environmental exposure to potential sources of benzene and other hydrocarbons; a case-control study.

AIM: To analyse the association between potential environmental exposure to hydrocarbons and the risk of acute childhood leukaemia. METHODS: A hospital based multicentre case control study, stratified on centre, age, and sex, with 280 leukaemia cases and 285 controls was carried out. Data were collected by a standardised interview of the mothers. RESULTS: No clear association was seen between maternal occupational exposure to hydrocarbons during pregnancy and leukaemia, or between residential traffic density and leukaemia. There was an association between dwellings neighbouring a petrol station or a repair garage during childhood and the risk of childhood leukaemia (OR 4.0, 95% CI 1.5 to 10.3), with a duration trend. The association, which appeared particularly strong for acute non-lymphocytic leukaemia (OR 7.7, 95% CI 1.7 to 34.3), was not altered by adjustment for potential confounding factors. CONCLUSIONS: Results showed an association between acute childhood leukaemia and dwellings neighbouring auto repair garages and petrol stations, which are benzene emitting sources. These findings could be due to chance, although the strength of the association and the duration trend are arguments for a causal association.

[Idiopathic hypereosinophilic syndrome: a case report in an infant]. / Le syndrome d'hyperéosinophilie essentielle: à propos d'un cas chez un nourrisson.

UNLABELLED: We report a case of idiopathic hypereosinophilic syndrome in a young child with favorable outcome after treatment with alpha-interferon. CASE REPORT: A 5-month-old boy presented with major eosinophilia (187 G/l) associated with splenomegaly. There was no evidence for parasitic or allergic disease. Acute leukemia was suspected but bone marrow smear and medullary caryotype were not compatible. Idiopathic hypereosinophilic syndrome was thus diagnosed. Corticotherapy was started and failed. Finally, complete remission was obtained with alpha-interferon treatment. CONCLUSION: Idiopathic hypereosinophilic syndrome is uncommon in children. Significant complications like cardiac dysfunction or hematologic malignancies can occur. Treatment has to be quickly started, in order to reduce eosinophilia. Haematological and echocardiographic follow-up are required.

[Dental anomalies following anticancer chemotherapy]. / Anomalies dentaires après chimiothérapie anticancéreuse.

Chemotherapy administered during childhood may induce dental abnormalities, such as acquired amelogenesis imperfecta, microdontia, hypodontia and altered root morphology. The magnitude of the defect varies according to the cytotoxic agents, the duration of their use and the stage of tooth development at the time of chemotherapy. Patients who received high-dose chemotherapy before the age of 5 are particularly concerned. The dental supervision of these children is based upon three orthopantomograms: the first one has to be performed before starting chemotherapy and will be used as a reference; the second is done soon after the drug therapy in order to evaluate the first consequences; the third is performed after the eruption of all permanent teeth (age 12-13 in average) in order to determine the dental abnormalities. In case of hypodontia, orthodontic treatment must be considered, but it is necessary to take into account the fact that it may increase the risk of root resorption. Preventive dental care is important for these children. It involves meticulous oral hygiene and frequent dental visits to assess and maintain dental health.

Gastrostomy as a decompression technique in children with chronic gastrointestinal obstruction.

BACKGROUND: Percutaneous or surgical insertion of gastrostomy tubes for feeding children has been well described. However, there is no report of percutaneous gastrostomy for chronic decompression in children with chronic gastrointestinal obstruction. The objective of this study was to evaluate this technique. METHODS: Eighteen gastrostomies were performed for gastrointestinal decompression in children. The patients ranged in age from 2 to 125 months (median: 15.5), and all had prolonged hospital stays for severe disease: severe gastroparesis and/or duodenal dysmotility (n = 8), duodenal stenosis (n = 2), chronic intestinal pseudo-obstruction (n = 4), enterocolitis (n = 2), and metastatic abdominal carcinomatosis (n = 2). The duration of symptoms before gastrostomy placement ranged from 0.5 to 44 months (median: 2), with major symptoms including epigastric pain, early satiety, nausea, vomiting, and bloating. RESULTS: The goals of gastric decompression and removal of the nasogastric tube were achieved in all patients, and all had significant relief of both nausea and emesis. Oral intake of liquids and soft foods was possible in 17 of 18 of the children. The tubes were kept in place for a median of 22.5 months (range, 2-73). There was neither long-term morbidity nor mortality associated with the presence of the tube. Seventeen patients returned home with gastric decompression and cyclic parenteral nutrition, two of them for terminal care. Six patients died. In all patients, gastrostomy was used throughout and did not contribute to the death of the patients. For seven children, tubes were removed because of resolution of small bowel or gastric outlet obstruction. CONCLUSIONS: Gastrostomy is an efficient and well-tolerated method of achieving long-term gastric decompression in children with abdominal obstruction not amenable to surgery and/ or resistant to medical treatment.

Nitrous oxide sedation in pediatric patients undergoing gastrointestinal endoscopy.

BACKGROUND: The ideal medication to administer to children before gastrointestinal endoscopy procedures has yet to be found. The efficacy of using inhaled nitrous oxide during endoscopy in children was assessed in a pilot study. METHODS: Patients aged 5 to 17 years, referred to our hospital for diagnostic upper gastrointestinal endoscopy or rectosigmoidoscopy procedures, were eligible for enrollment in this study. All received 50% nitrous oxide in oxygen (Entonox; AGA, Rueil-Malmaison, France) before endoscopy and some of them again during endoscopy. The pediatric endoscopist and the nurse performing the procedure were separately asked to rate cooperation, emotional state, drowsiness and overall efficacy of sedation. Oxygen saturation and adverse effects were recorded throughout the procedure. After endoscopy, children scored their degree of pain during the procedures on a visual analog scale (0, no pain; 100, agony) and on a body outline (projective method). Any adverse effects were noted. RESULTS: Thirty-seven patients were enrolled in the study. Thirty patients underwent diagnostic upper gastrointestinal endoscopy and seven diagnostic rectosigmoidoscopy. The median time from administration of nitrous oxide until insertion of the endoscope was 5 minutes (range, 3-8 minutes). Good or excellent efficacy of the sedation was noted in 92% of children by the endoscopist and in 89% by the nurses. Good or excellent cooperation was noted in 92% of the children by the endoscopist and in 78% by the nurses. The children's pain score on the visual analog scale ranged from 5 to 100 (median, 20) for upper gastrointestinal endoscopy and from 0 to 30 (median, 0) for rectosigmoidoscopy. The time of discharge after endoscopy, defined as the time elapsed between the end of the endoscopy and discharge from the endoscopy suite, varied from 1 to 7 minutes (median, 1.5 minutes). CONCLUSION: Entonox provides rapid and effective analgesia without heavy sedation, leads to adequate relaxation and cooperation, and facilitates quick and efficient endoscopy. The effect of Entonox was of short duration, allowing the children to leave the endoscopy unit without need for a long recovery period. The adverse effects of Entonox appeared to be minor, and their duration was always brief. Nitrous oxide-oxygen inhalation may provide a valuable alternative to conventional sedation regimens during gastrointestinal endoscopy in children, but randomized and prospective studies comparing nitrous oxide sedation and conventional sedation regimens are necessary.

[Uncommon etiology of extradural hematoma]. / Etiologie inhabituelle d'un hématome extra-dural.

Intracranial or spinal epidural hematomas are almost always of post-traumatic origin, and rarely related to rupture of a dural vascular malformation or a hemostasis disorder. We report a case of spontaneous cerebral epidural hematoma observed in a young girl with homozygous sickle cell disease. After analysis of post-operative cerebral MRI, showing skull bone infarction inside and around the bone flap, it was considered that the skull bone infarction could be responsible for a diploic venous thrombosis with subsequent "spontaneous" epidural hematoma.

[Bone marrow monosomy 7 in children]. / Monosomie 7 médullaire chez l'enfant.

Three new cases of monosomy 7 are described. Two children, before onset of overt leukemia, had a preleukemic state: one with thrombopathy and myelodysplastic syndrome, the other with a moderate splenomegaly and an absolute monocytosis. In these two cases the leukemia was chemoresistant. The last child had a subacute myelomonocytic syndrome (juvenile type of chronic myelogenous leukemia) without high fetal hemoglobin value. She died from cachexia. The poor prognosis of monosomy 7 is underlined and such a chromosome deletion should be searched in myeloproliferative syndrome with monocytosis.

[Graft versus host reaction following materno-fetal transfusion. Apropos of 1 case]. / Réaction du greffon contre l'hôte après transfusion materno-foetale. A propos d'une observation.

After GVHR by probable maternal-foetal in utero transfusion, a male infant carrying a serious combined immune deficit (DICS) has presented a second post transfusional GVHR. On the occasion of this observation, the authors recall the main signs which permit to evoke this rare diagnosis as early as possible, as well as some main preventive measures that can be applied in current practice.