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Biomedical subjects

Y Perel

Publications and source records attributed to Y Perel.

At least 55 records · Page 3Linked to original sources
PubMed1997

N-Myc gene amplification is a major prognostic factor in localized neuroblastoma: results of the French NBL 90 study. Neuroblastoma Study Group of the Société Francaise d'Oncologie Pédiatrique.

PURPOSE: To assess the relevance of N-Myc gene amplification (NMA) as a prognostic factor in localized neuroblastoma (NB) and to evaluate whether less intensive adjuvant treatment is advisable in infants without NMA. PATIENTS AND METHODS: Assessment of NBs included clinical and imaging data to allow tumor-node-metastasis (TNM) staging, biologic determinations (N-Myc gene analysis), and standard histology and work-up to eliminate metastatic spread (metaiodobenzylguanidine [MIBG] scintigraphy and extensive bone marrow staging). Resectability was defined according to imaging findings. Chemotherapy was indicated in children older than 1 year at diagnosis who had postoperative residual disease or lymph node (LN) involvement, in infants with NMA, or as primary treatment in children with an unresectable NB, including dumbbell tumors. Radiotherapy was recommended in children older than 1 who presented with persistent gross residual disease at the end of therapy. RESULTS: Between 1990 and 1994, 316 consecutive children who presented with a localized NB were registered in the NBL 90 study. The median age was 12 months, and 42 patients had dumbbell tumors (13%). NMA was found in 22 of 225 assessable children (10%) and correlated with adverse prognostic indicators such as age older than 1 year, an abdominal primary tumor, a large tumor (T3), and unresectability. Among 186 children who had primary excision, five died of surgery-related complications. Primary chemotherapy was given to 130 patients, which allowed removal of the tumor in all but four. The 5-year overall survival (OS) and event-free survival (EFS) rates were, respectively, 91% and 84% with a median follow-up time of 36 months. The outcome of infants and older children was similar (P = .2). EFS of patients with resectable tumors was slightly better than with unresectable primary tumors (EFS, 89% v 78%; P = .02). In dumbbell NBs, neurologic recovery was achieved in 74% of cases that presented with symptoms, and initial laminectomy was avoided in 75% of children. In a univariate analysis, large tumors, high neuron-specific enolase (NSE) and lactate dehydrogenase (LDH) levels, positive LNs, macroscopic residue, and NMA adversely influenced outcome. In the multivariate analysis, NMA was the most powerful unfavorable predictive indicator: OS and EFS rates for these children were 36% and 32%, compared with 98% and 90% in nonamplified tumors (P < .001). CONCLUSION: Our data confirm the overall good prognosis of localized NBs, even when unresectable. NMA is the most relevant adverse prognostic factor in localized NBs, and more intensive treatment should be investigated in these patients. Prospective studies of other biologic factors are warranted to tailor therapy more accurately. The EFS of children who underwent primary surgery was excellent, and further justifies elimination of adjuvant treatment provided they have no NMA. Despite the elimination of postoperative therapy, infants with non-NMA tumors have an excellent outcome, which suggests that initial chemotherapy can be further reduced in case of unresectable NBs.

Adolescent↗
PubMed1997

Philadelphia negative BCR-ABL positive chronic myeloid leukemia mimicking juvenile chronic myeloid leukemia in a 2-year-old child.

We present the case of a two-year-old child with an atypical presentation of chronic myeloid leukemia. At diagnosis, he showed clinical and biological features of juvenile chronic myeloid leukemia (CML). However, eosinophilia was observed in blood and bone marrow. The bone marrow karyotype did not demonstrate the Philadelphia chromosome but BCR-ABL rearrangement was shown to be present by reverse transcriptase polymerase chain reaction (RT-PCR) analysis and confirmed by fluorescent in situ hybridization (FISH) analysis. Discussion centres on the differentiation between juvenile CML and childhood chronic myelogenous Leukemia and the importance of carrying out RT PCR for all juvenile CML cases.

Child, Preschool↗
PubMed1996

Induction chemotherapy followed by allogeneic bone marrow transplantation or aggressive consolidation chemotherapy in childhood acute myeloblastic leukemia. A prospective study from the French Society of Pediatric Hematology and Immunology (SHIP).

In the LAME89/91 protocol, children with acute myeloid leukemia (AML) who achieved complete remission (CR) after induction chemotherapy, were treated either with allogeneic bone marrow transplantation (BMT) if they had an HLA-compatible related donor or with chemotherapy including high-dose cytarabine. The objectives of this study were to describe the overall results of this strategy and to compare the two post-remission arms. Two hundred and thirty-one children were enrolled in the protocol. Induction chemotherapy consisted of a combination of cytarabine and mitoxantrone. A CR was achieved in 204 children (88%).

Acute Disease↗
PubMed1996

Allogeneic bone marrow transplantation vs aggressive post-remission chemotherapy for children with acute myeloid leukemia in first complete remission. A prospective study from the French Society of Pediatric Hematology and Immunology (SHIP).

The objective of this study was to compare allogeneic bone marrow transplantation (BMT) with high-dose cytarabine containing chemotherapy in children with acute myeloid leukemia (AML) in first complete remission (CR). One hundred and seventy-one children were enrolled on the LAME89/91 protocol. Induction chemotherapy was a combination of cytarabine and mitoxantrone. After achieving CR, patients who had an HLA-identical sibling donor underwent allogeneic BMT. Children not eligible for BMT received post remission chemotherapy which included two consolidation courses, the second consolidation consisting of high-dose cytarabine with amsacrine and asparaginase. CR was achieved in 149 children (87%). Thirty-two had an HLA-identical sibling donor and were eligible for BMT. These 32 patients, as well as an additional child who had a one antigen HLA-mismatched father, received BMT during first CR. Consequently, 33 patients were analyzed in the BMT group and 116 in the chemotherapy group. The 4-year probability of relapse was 26 +/- 15% in the BMT group and 47 +/- 10% in the chemotherapy group (P = 0.04). The risk of therapy-related death was 3% for BMT and 7.7% for chemotherapy. Disease-free survival (DFS) was 72 +/- 15% in the BMT group and 48 +/- 10% in the chemotherapy group (p = 0.02). We conclude that allogeneic BMT from a matched sibling donor is the treatment of choice for reducing the relapse risk and for increasing DFS in children with AML in first CR.

Amsacrine↗
PubMed1996

[Acute torsion of an accessory spleen].

A case of acute torsion of an accessory spleen is reported. In a 15-year-old girl was admitted to hospital with acute abdominal pain. Ultrasonography showed a round, hypoechoic, solid mass. Computed tomography demonstrated a low-density mass with peripheral enhancement after intravenous contrast medium. These findings suggest the presence of a capsule. At laparotomy, the patient was found to have torsion and infarction of an accessory spleen. Sonographic and computed tomographic findings are discussed.

Acute Disease↗
PubMed1995

Infantile myofibromatosis.

An unusual case of infantile myofibromatosis with foci of calcifications was presented, with atypical age of onset and location. The radiological features of this tumor are not always evocative, and histological examination is necessary for diagnosis.

Actins↗
PubMed1993

Curability of relapsed childhood B-cell non-Hodgkin's lymphoma after intensive first line therapy: a report from the Société Française d'Oncologie Pédiatrique.

The very high cure rate in advanced B-cell non-Hodgkin's lymphoma in children using intensive multiagent therapy has been previously reported by the French Sociéte Française d'Oncologie Pédiatrique lymphoma Malin B type (LMB) group. To address the issue of salvageability in an unselected group of patients who had all received the same front-line therapy, the outcome of relapses following the LMB 84 (216 patients) protocol have been reviewed. Fourteen percent of patients achieving complete remission (CR) relapsed, ie, 27 of 195. Relapse sites comprised the central nervous system (CNS) alone (6 cases), lung or mediastinum (2 cases), abdomen (8 cases), head and neck (2 cases), or multifocal (9 cases). There were three early deaths due to disease. Twenty-four patients received rescue chemotherapy regimens and 15 were treated with high-dose chemotherapy and bone marrow rescue (1 allogeneic). Of these, 9 were in second CR, 4 in second partial remission, and 2 treated during progressive disease. One died in CR from treatment-related toxicity. Ten relapsed postbone marrow transplant and 4 are alive disease free and probably cured. Two of the long-term survivors had some delay during initial chemotherapy due to toxicity and two were isolated CNS relapses. Twelve of 27 patients did not proceed to megatherapy (12 of 12 died).

Bone Marrow↗
PubMed1993

Combined continuous infusion etoposide with high-dose cyclophosphamide for refractory neuroblastoma: a phase II study from the Société Française d'Oncologie Pédiatrique.

PURPOSE: Patients older than 1 year with stage IV neuroblastoma who fail to achieve complete remission (CRem) have a particularly poor long-term prognosis. In an attempt to improve the outcome of these refractory patients, we tested a new drug combination. PATIENTS AND METHODS: Twenty-nine children with advanced neuroblastoma (27 stage IV and two stage III) were entered onto this phase II study. All were refractory to conventional chemotherapy and had measurable disease at the time of the trial. The regimen was a combination of high-dose cyclophosphamide (2 g/m2/d) on days 2, 3, and 4, and etoposide (VP16; 50 mg/m2/d) by continuous intravenous (IV) infusion on days 1 to 5. A pharmacokinetic study of VP16 was conducted in eight patients to determine whether the goal of persistent plasma levels between 1 and 5 micrograms/mL was achieved. RESULTS: Patients received a median of two courses, for a total of 58 courses. The median interval between each course was 32 days. In the 28 assessable patients, the overall response rate was 43%, with one CRem and 11 partial remissions (PRems). No life-threatening complication was observed in these heavily pretreated patients. The median duration of neutropenia (< 5 x 10(9)/L) was 14 days, and that of thrombocytopenia (< 50 x 10(9)/L) was 11 days. The overall incidence of sepsis was 27%. Gastrointestinal toxicity was frequent, but mild. Electrolyte disturbance with antidiuretic hormone (ADH)-like syndrome occurred in eight courses, but resolved rapidly. Grade > or = 2 hemorrhagic cystitis was observed in three courses. No cardiac toxicity was observed. There were no treatment-related deaths. Pharmacokinetic analysis showed that mean steady-state plasma levels (Css) of VP16 were greater than 1 microgram/mL during all the courses. CONCLUSION: This new drug combination appears to be effective in advanced neuroblastoma. Its toxicity remains manageable, with no life-threatening complications. Further evaluation in patients with less-advanced disease is warranted.

Adolescent↗
PubMed1993

[Cutaneous manifestations of Kawasaki disease. Apropos of 30 cases].

A series of 30 cases of Kawasaki disease has been studied retrospectively over a period of 11 years. The aim was to reassess the diagnostic value of the dermatological manifestations. A modification of the extremities was observed in 28 patients (23 had early inflammatory lesions, 25 had late desquamation). Exanthema was constant, polymorphous and most often urticaria-like. Vesicles, pustules or purpura were noted during the course of the eruption in 7 patients. A perineal eruption was observed in 17 cases and was found of good diagnostic value even though not pathognomonic. Cheilitis was the most frequent of buccopharyngeal modifications (93 p. 100). Conjunctival hyperemia was noted in 26 patients. Eight children had cardiovascular complications. Among these cases, the modification of the extremities seemed to be more pronounced and stomatitis and arthritis were apparently more frequent. Most of all, the inflammatory syndrome was significantly more severe as concerns CRP and polymorphonuclear leukocytes counts. Dermatological examination often rules out other diagnoses, such as measles, scarlet fever and staphylococcal toxic shock syndrome. However, a complete etiological workup remains mandatory.

Cheilitis↗
PubMed1993

Alveolar soft part sarcoma. A rare tumor of unusual evolution in pediatrics.

PURPOSE: A case of alveolar soft part sarcoma of the arm with metastatic pulmonary miliaria is reported in a 12-year-old girl. RESULTS: Although the size of the metastases increased greatly and progressively for 9 years with no improvement under chemotherapy, the patient's general condition remained good. CONCLUSIONS: The course in pediatric oncology of this rare mesenchymatous tumor is very unusual; the histologic pattern is characteristic but the histogenesis remains unclear. The treatment of choice is tumoral excision. Other therapies involving secondary deposits are far from satisfactory, and the prognosis is poor because of the high rate of metastases.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1992

Perineal ecthyma gangrenosum in infancy and early childhood: septicemic and nonsepticemic forms.

BACKGROUND: Ecthyma gangrenosum is characterized by necrotic ulcerations surrounded by an erythematous halo. It is secondary to Pseudomonas aeruginosa infection. Most lesions are located in the anogenital and axillary areas, but the route of infection is generally difficult to establish. OBJECTIVE: We report six children with perineal ecthyma gangrenosum and discuss predisposing factors, origin, and route of infection. METHODS: This was a retrospective clinical study. RESULTS: Three children had blood cultures positive for P. aeruginosa, and one died. Predisposing factors were present in all cases; two had received chemotherapy (neuroblastoma, acute lymphoblastic leukemia), and two had idiopathic granulocytopenia. The last two patients previously had received treatment with systemic antibiotics and had abnormal granulocyte killing several months later. CONCLUSION: Septicemic ecthyma gangrenosum can be rapidly fatal in young children and requires aggressive antibiotic therapy. Benign ecthyma gangrenosum in healthy infants may result from a modification of bowel microflora after antibiotic therapy in conjunction with maceration in the diaper area. However, careful evaluation and long-term follow-up must be done to detect neutropenia, functional abnormalities of granulocytes, or a possible immune deficiency.

Child, Preschool↗
PubMed1992

[Infantile spasms associated with a brain tumor].

BACKGROUND: Infantile spasms are exceptionally associated with a focal cerebral lesion such as a brain tumor. CASE REPORT: A 15 month-old girl was treated with hydrocortisone and valproic acid for infantile spasms that had appeared a few days earlier. Her psychomotor development was considered normal and the CT scan was normal. Clinical symptoms disappeared within 10 days and the EEG became normal except for a few epileptiform discharges in the frontal areas. The girl developed partial seizures at the age of 5 years. Imaging techniques showed a brain tumor in the right frontal lobe. Surgical excision was followed by radiation therapy. Analysis of the tumor showed an astrocytoma. The girl is normal 18 months after intervention. CONCLUSION: It is difficult to determine a relationship between the infantile spasms and the brain tumor in this case. The moderately asymmetrical pattern of the EEG after infantile spasms, the fact that these spasms appeared relatively late and the persistence of discharges, lead to repeated specialized investigations.

Astrocytoma↗
PubMed1992

[Beta-ketothiolase deficiency: a case of ketoacidosis with hyperglycinemia].

Regarding a case of beta-ketothiolase deficiency revealed by ketoacidosis with hyperglycinemia, the authors show the way to diagnose and to treat this disease. Ketoacidosis without hyperglycemia or lactacidemia suggested this diagnosis. Gas chromatography-mass-spectrography revealed unusual urinary excretion of metabolic products of isoleucine. The enzymological study of fibroblasts confirmed the diagnosis. The treatment of acute episodes consisted of acidosis control and exclusive glucides intake before diagnosis. Afterwards, a controlled proditic diet and L-carnitine must be given and fasting must be avoided.

Acetyl-CoA C-Acyltransferase↗
PubMed1992

[Cardiac failure by major arterial hypertension secondary to nephroblastoma].

The authors report on the case of a 7 week-old boy, in whom a renal mass was discovered after general symptoms were observed. Within 48 h, cardiac failure secondary to systemic arterial hypertension occurred, requiring intensive care. After a few days of mechanical ventilation and alternating elevated and low blood pressure, improvement was obtained with captopril and frusemide enabling further investigations to be carried out which lead to the diagnosis of Wilms tumor. During left-sided nephrectomy, elevated renin from the left renal vein was found. The post surgical course was excellent. Several authors have reported on the association between arterial hypertension and nephroblastoma as being the result of hyperreninism due to hilar compression; however severe hypertension was uncommon. Renin activity determination from the tumoral tissue had led to a different interpretation, ie primary hyperreninism: in the case of mesoblastic nephroma, only the non tumoral but compressed tissue contains a large quantity of renin; in the case of nephroblastomas, only the tumoral tissue contains renin. The question now is whether all or only certain nephroblastomas secrete renin.

Heart Failure↗
PubMed1992

[Gangrenous ecthyma of the diaper area in infants].

Ecthyma gangrenosum due to Pseudomonas aeruginosa is a skin infection in which necrotic ulcerations surrounded by a red areola develop. The diaper area is the region most often involved in infants. Typically, ecthyma gangrenosum occurs in patients with septicemia and risk factors (chemotherapy, neutropenia). However, transient bacteremia or an infection confined to the skin may be the cause in some patients, with maceration in the diaper area and previous antibiotic therapy as risk factors.

Diagnosis, Differential↗