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Rhabdomyosarcoma is an extremely virulent rare tumor whose early diagnosis considerably improves survival and visual prognosis. We report the case of a 2-year-old child with levator palpebrae superioris muscle rhabdomyosarcoma revealed by a sudden and isolated blepharoptosis. Initially, clinical and imaging investigations did not show any abnormality but a painful tumor with some hematoma quickly developed, so the investigations were repeated. The CT-scan showed an extra-conal tumor that had developed at the superior part of the orbit. Histology confirmed the diagnosis of embryonic rhabdomyosarcoma. Because of its results, treatment consisting of chemotherapy associating ifosfamide, vincristine, actinomycin and orbital radiotherapy of 40 Gy with a local addition of 10 Gy were administrated with successful results after a 3-year-follow-up.

The optic nerve glioma is a relatively rare pathology in children and only a few cases have been described in infants. This paper reports the symptoms of this exceptional case in a 4-month-old child and reviews the differential diagnoses at this age. Finally, we discuss the diagnosis and therapeutic possibilities.

The immunomodulatory effect of extracorporeal photochemotherapy (photopheresis) coupled with its efficacy in lymphocytic skin diseases provides a rationale for its use for erosive lichen planus. We report two cases of chronic oral erosive and corticoresistant lichen planus successfully treated with photopheresis.Case 1. A 61-years-old man had erosive oral lichen planus for four years. Oral steroids were contra-indicated due to iatrogenic pancreatitis. After nine photopheresis sessions, subjective improvement occurred and the oral lesions were stabilized. Case 2. A 17-years-old woman presented corticodependent oral and genital erosive lichen planus and cutaneous lesions. After seven sessions of photopheresis, the patient was able to eat again and the pain decreased. After 20 sessions, the cutaneous lichen planus disappeared and complete remission of the mucous lesions was obtained without corticotherapy.These two cases, together with 23 other cases reported elsewhere, strongly suggested the value of photopheresis in the treatment of erosive lichen planus. However, relapses after treatment withdrawal appear extremely frequent.

In 10 years, the neonatal autoimmune enteropathy has been individualized from other causes of neonatal severe protracted diarrheas as a syndrome called Ipex for Immunodysregulation, Polyendocrinopathy, Enteropathy, X-linked. Thanks to linkage analyses in affected families, this rare paediatric syndrome with fatal outcome has been correlated to mutations of the foxp3 gene. Homozygous loss of function of foxp3 gene results in the absence of development of a crucial subpopulation of lymphocytes with CD4+CD25+ phenotype, called regulatory T-cells. The study of these lymphocytes allows a better understanding of the immune system homeostasis and of the physiopathology of Ipex syndrome, which is a prerequisite for treatment. Achieving ex vivo manipulation of such lymphocytes will end up on promising applications of cell therapy.

The ability of two commercially available diagnosis rapid assays in detecting rotavirus antigen was compared in a prospective study conducted from September 2002 to May 2003. Five hundred and twelve faecal specimens were studied by IDEIA Rotavirus enzyme immunoassay test (EIA) and Diarlex MB immunochromatographic test (ICG). Specimens giving discrepant results were examined by electron microscopy (EM) and clinical data reconsidered. Out of 512 stool specimens, 155 (30.3%) were positive and 332 (64.8%) negative with the two assays. Discrepant results were obtained for 25 (4.88%) specimens (24 children, 1 adult), with EIA giving more positive results. The retrospective examination by EM, possible for fifteen stools on the 25 that gave discrepant results, confirmed the presence of rotavirus in 7/14 stools which were positive only by EIA and in the stool specimen that was found positive only by ICG. The 25 clinical observations re-examination showed the presence of GEA signs in all cases. The statistical analysis shows an excellent concordance between the EIA and the ICG tests (kappa = 0.89, IC(95%) = [0.85-0.93]) in spite of the underestimation of ICG test in comparison with EIA test (P0.0001).

Scleromyxedema is the consequence of a dermal infiltration by mucine, commonly associated with a monoclonal gammapathy of unknown significance.A 46 year-old woman was hospitalized for a scleromyxedema with a bilateral macular edema and a restricted pulmonary syndrome. A quite complete cutaneous response and a complete ocular and pulmonary response were obtained after 12 extracorporeal photopheresis courses and 4 flashes of prednisolone (17 months follow-up).There is no consensus on guidelines for the treatment of scleromyxedema. Steroids and melphalan are usually indicated. However these drugs induce severe side-effects. In the absence of controlled studies concerning the efficiency of the different drugs used in scleromyxedema, the main advantage of photopheresis lies in the safety of this procedure.

Gastric tumours are exceptional in children and are usually discovered due to digestive tract bleeding or after palpation of a mass in the abdomen. Appropriate exploratory techniques are need to correctly localize the lesions in the stomach. Nevertheless, the absence of any particular signs can mask the polymorphic nature of the lesions that can only be determined by pathologic examinations. The extent of the surgical exeresis and prognosis depend on the nature of the pathological process.

78 consecutive cases of PCP have been analysed: 59 (75.6%) cases were associated with HIV infection and 19 (24.4%) cases were associated with solid tumors and hematologic malignancies at advanced stage of the disease and renal transplantation. It could suggest primary prevention for such patients.

From August 1985 to April 1988, 17 patients have been allografted in France for TM. Fourteen (82%) are alive, 10 (58%) are cured and 4 in autologous reconstitution. Three died, 2 of whom after a second transplantation, and 2 due to CMV interstitial pneumonia. Ten of 17 were conditioned by cyclophosphamide (Cy: 200 mg/kg) and busulfan (Bu: 14 mg/kg): 10 are alive, 6 cured and 4 in autologous reconstitution. Five received Cy (200 mg/kg) Bu (16 mg/kg) +/- total lymphoid irradiation (TLI) (3 patients): 2 patients are cured (one after hepatic veno-occlusive disease), and 3 died. For 2 patients, conditioning included total body irradiation: both are cured. Graft versus host (GVH) reaction prevention included: 4 T cell depletion (with additional prevention in 3; 2 are cured, one is in autologous reconstitution, 1 died); 5 cyclosporine alone (2 cured, 2 autologous reconstitution, 1 dead) and 8 cyclosporine + methotrexate (6 cured, 1 autologous reconstitution, 1 dead). Four of 12 evaluable patients presented a less than or equal to 2 grade acute GVHd. None had received a T cell depleted marrow. Bone marrow transplantation is the only curative treatment currently available for TM patients with HLA identical donors. It should be proposed to any young patient before iron overload is established. Optimal conditioning includes Cy (200 mg/kg) + Bu (14 mg/kg). The best protocol for GVHd prevention remains to be defined.

An 11 year-old boy was treated for acute promyelocytic leukemia (ALM3) with chemotherapy according to the LAME 86 protocol (aracytin and rubidomycin). The first complete remission was consolidated by two autologous bone marrow transplantations. Following autologous graft, a persistent thrombocytopenic purpura appeared. Autoimmune origin was indicated by a decrease in platelet survival time and by the presence of high levels of antiplatelet antibodies.

Two brothers presented with hemophagocytic reticulosis with neonatal onset. Early clinical and biological symptoms included pallor, hepatosplenomegaly and anemia, thrombocytopenia. Evolution was lethal in both cases, at 5 months and 13 days of age, respectively. Diagnosis was confirmed in both by the pathologic findings: diffuse lymphohistiocytic cellular proliferation with hemophagocytosis and atrophy of the lymphoid tissue. The diagnostic difficulties of this disease in the neonatal period are emphasized.

A case of extraskeletal Ewing's sarcoma arising in right lumbar region with involvement of epidural space is described in a 16 years old girl. The diagnosis of Ewing's sarcoma was done from cytochemical and electron microscopic studies which showed intra-cellular glycogen granules whereas cytologic and immunological analysis eliminated lymphoid malignancies. This case emphasized the interest of these techniques, adjunctive to routine light microscopic examination for the pursuit of a specific diagnosis in the small round cell neoplasm.

Twenty-six children with acute leukemia were treated with allogeneic marrow transplantation from HLA identical siblings after a conditioning regimen with Cyclophosphamide-total body irradiation (19 patients), Melphalan-total body irradiation (6 patients) or Busulfan-cyclophosphamide (1 patient). Eighteen were transplanted in complete remission (4 with acute non lymphoblastic leukemia in first remission, 14 with acute lymphoblastic leukemia: 6 in first, 6 in second and 2 in subsequent remission): 2 died of cytomegalovirus pneumoniae, 1 relapsed and 15 survive in continuous complete remission from 5 to 42 months after transplantation (median = 22 months). Eight were transplanted in relapse, 7 achieve complete remission, 5 of them relapsed, 1 died of G.V.H. and 1 survives in continuous complete remission 46 months after transplantation. Actuarial analysis shows a disease free survival rate at 3 years of 82% for patients transplanted in remission and 12% for patients transplanted in relapse (p less than 0.01).

The case of an infant with bone marrow dysfunction and exocrine pancreatic deficiency is reported. Bone marrow dysfunction presented at birth, with a refractory sideroblastic anemia later associated with neutropenia and thrombocytopenia. Erythroid and myeloid precursors had a marked cytoplasm vacuolization and very poor in vitro growth. The exocrine pancreatic deficiency was shown by the pancreozymin-secretin stimulation test and by the study of fat digestion. This case report is different from Shwachman's syndrome, but similar to a syndrome of unknown etiology, recently described by Pearson in 4 children. The normality of immune investigations and of the culture of T lymphocyte precursors, in our patient, shows that the bone marrow dysfunction spares the lymphoid lineage. The simultaneous occurrence of bone marrow and pancreatic cells dysfunction suggests either a process acquired during embryonic life, or a gene mutation with pleiotropic effects.

Infection is the most important cause of mortality in leucopenic patients. A broad spectrum antibiotic therapy is imperative in febrile and neutropenic patients. In a multicentric study we have used ceftazidime (100 mg/kg/d) and netilmicin (6 mg/kg/d) in 88 children (fever greater than or equal to 38.5 degrees C, neutropenia less than 500/mm3) treated for acute leukemias (59), non Hodgkin lymphomas (13) or solid tumors (16). Median age was 7 years (2 months-16 years). In patients who continued to remain febrile, vancomycin (40 mg/kg/d) was added after 48 hours. The effective treatment was continued until a neutrophil count greater than 1,000/mm3. The first combination (ceftazidime + netilmicin) was effective in 64 children (73%) and the second combination (ceftazidime + netilmicin + vancomycin) in 11 patients. Bacteria were isolated in 39 children: Escherichia coli: 9, Staphylococcus epidermidis: 9, Staphylococcus aureus: 8, Streptococcus: 6, Pseudomonas aeruginosa: 3, Streptococcus pneumoniae: 1, Haemophilus: 1, Klebsiella pneumoniae: 1, Proteus: 1, Serratia: 1, Flavobacterium: 1. In these 39 patients, 30 became apyretic with ceftazidime and netilmicin and 6 after vancomycin. All blood culture were negative after the first combination. The median duration of antibiotic therapy was 14 days (5-9 days: 28, 10-20 days: 43, greater than 20 days: 17). There were no death, no superinfection. Tolerance was good without kidney or liver or biological perturbation. We conclude that the combination ceftazidime and netilmicin is effective in neutropenic children.

Thirteen episodes of fever in bone marrow transplantation recipients (23 months to 11 years old children) were treated by ceftazidime (100-200 mg/kg/j) and netilmicin (7 mg/kg/j). Vancomycin was added at the 24th hour in 10 cases of persistent fever. 6 presumed agents of infection were isolated before antibiotic treatment: blood cultures (streptococci 2, staphylococcus 1, proteus 1), fecal sample (E. coli 1), urine (E. coli 1). Modifications of aerobic fecal flora were studied under this treatment. E. coli, staphylococci and enterococci were the mainly strains isolated. There were no third generation cephalosporins resistant Gram-negative bacteria. High level resistance to aminoglycosides was observed in enterococcal strains, isolated during and after treatment. Ceftazidime-netilmicin (+/- vancomycin) was an effective and safe combination for the management of febrile neutropenic episodes.

A case of olfactory neuroblastoma in a 9 month-old girl is reported. Blindness and purulent meningitis were the first symptoms. Radiological examinations including CT scan revealed a sphenoidal tumor with penetration of the meninges. Surgery revealed an ethmoido-sphenoidal tumor mass extending into the pituitary fossea which consisted of malignant cells with the histological aspect of esthesioneuroblastoma. The rarity of this tumor in early childhood and its clinical polymorphism are emphasized.

We observed a case of lung infection with Paragonimus for a 4 year old child in Gabon. The treatment with bithionol was partially uneffective. Indeed, after the end of this treatment we observed hypereosinophilia, hyper IgG and X Rays pulmonary opacities but without eggs in stools of sputum.

A 13 year-old boy presented with Burkitt's type acute lymphoblastic leukemia (B-ALL). Studies of immune markers on his lymphoblasts suggest they have reached a fairly advanced level of differentiation with in the B-cell lineage. Presence of 1q+ and 8q- chromosomes suggest an unusual (1;8) translocation and is in agreement with current concepts concerning the frequent chromosome 8 changes in B-ALL.

Since 1983, the authors have performed a surgical ligation of the splenic artery in 7 patients suffering hypersplenism due to hematological diseases (4 Minkowski-Chauffard, 2 Betathalassemia Major, 1 Idiopathic Thrombocytopenic purpura). The immediate post operative course and long-term outcome of the patients were evaluated on clinical and biological grounds. The early post operative period was not influenced by the underlying hematological disorder. In all patients the hemoglobin, erythrocyte and platelet counts significantly increased and were related to functional hyposplenism. Delayed outcome depended on the underlying disease. Patients with Betathalassemia returned to preoperative status within months; thus, the only advantage of the arterial ligation was to transiently decrease the requirements in blood transfusion. Two patients with Minkowski-Chauffard disease did not present further episodes of acute hemolysis and did not required additional blood transfusion. On the other hand, the other two patients with Minkowski-Chauffard disease progressively returned to preoperative status, including severe episodes of hemolysis, and required a splenectomy be performed. Since the arterial supply to the spleen remains inchanged after the operation, it may be expected that the results (i.e. long-duration hyposplenism) could be improved by performing extensively devascularization of the spleen to complete the ligation of the splenic artery.