Biomedical subjects
Publications and source records attributed to D Frappaz.
A 13 years old girl is admitted for severe chronic anemia. Few blood sac looking like lesion are discovered. A Blue Rubber Bleb Nevus Syndrome is confirmed discovery of multiple intestinal angioma. No deep lesion is discovered otherwise. Clinical characteristics, nosology and evolutive trend of this rare syndrome are recalled.
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Authors report the case of a 19 year old metastatic Ewing's sarcoma, prophylactically treated for suspected tuberculosis by Rifampicin and INH. Hepatic failure was induced by accidental overdose of INH. A high dose containing VCR protocol associated, lead to a coma with convulsions and severe motor peripheral neuropathy partially regressive. Neurological incidents of VCR, INH and their association are reviewed.
Neutropenia developed in 3 pediatrics patients during treatment with oxacillin. The time of onset ranged from 18 to 24 days after beginning treatment with dose of 150 mg/kg/24 h. Concomitant symptoms were fever and rashes. During treatment with oxacillin it is necessary to monitor blood cell count every week.
We studied the effects of prolonged pancuronium administration in 10 neonates under assisted ventilation for hyaline membrane disease. Pancuronium bromide was administered from the time of intubation with a load dose of 0.1 mg/kg followed by a continuous infusion of 0.03-0.07 mg/kg/h. Tolerance, especially cardiovascular, was excellent after 72 to 525 h of therapy (mean 173 h). Oxygen needs, incidence of mechanical complications and the general evolution of idiopathic respiratory distress were not significantly affected by muscle relaxation. The benefits of systematic pancuronium administration during respiratory intensive care in neonates with hyaline membrane disease appear to be of little value when considering the necessary constraints (corneal, cutaneous, vesical and tracheal supervision).
With one personal case and thirteen cases from literature about distal deletion of the long arm of chromosome 4 (4 q-), authors try to describe a clinical syndrome related to deletion of segment 4 q 31 leads to q ter. This syndrome includes a normal intrauterine growth, a growth and mental retardation. Morphological abnormalities consist in microcephaly, palato-cheiloschisis with micrognathia, hypertelorism with epicanthald folds, large nose bridge with anteverted nases, various anomalies of ears; clinodactily of Vth finger and toe, various cardiac defects. New banding techniques (prometaphase) have led to more precise delineation of break point, but this very distal deletion could not bring any new information for the gene mapping.
A 5,9 year old girl presented a severe and acute auto-immune hemolytic anemia, unresponsive to steroids, treated by 10 plasma exchanges, immunosuppressive agents and splenectomy. Despite an anticoagulant treatment, occurrence of multiple thrombosis. Unusual evolution of Mycoplasma serological tests.
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Neuroblastoma may present with spinal cord compression due to dumbbell extension of thoracic or abdominal disease. Isolated intraspinal involvement as the sole site of relapse is rare. Two infants with poor-prognosis stage IV neuroblastoma presented early after high-dose therapy and autologous bone marrow transplant with isolated spinal relapse heralded by misleading symptoms. The rarity of such isolated relapses is reviewed, and an etiologic hypothesis is proposed.
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This retrospective study compared the overall survival, the event-free survival, and the timing of chemotherapy in patients with advanced Burkitt lymphoma with and without laparotomy. Thirty-five patients with advanced abdominal Burkitt lymphoma treated at least partially at the Centre Léon Bérard between 1981 and 1992 were included in this study. The diagnosis was obtained by laparotomy (LAP group) in 21 patients (17 stage III, 4 stage IV) and by other methods (non-LAP group) in 14 patients (5 stage III, 9 stage IV). The overall survival (71 and 93%) and the event-free survival (66 and 79%) were similar in the LAP and non-LAP groups, and the relapse rate was five (three local) in the LAP group compared with three (none local) in the non-LAP group. The local complication rate (9 of 21 versus 2 of 14) and the toxic death rate (2 of 21 versus 1 of 14) were slightly higher in the LAP group. Laparotomy also caused delays in therapy and increased the overall hospital stay. The mean interval from diagnosis to the start of the fourth course of chemotherapy was 57 days compared with 48 days and the average hospital stay was 44.4 days compared with 39 days for the LAP and non-LAP groups, respectively. Because advanced Burkitt lymphoma can be diagnosed by fine-needle aspiration, and chemotherapy cures more than 80% of the patients, there is no need for initial surgery, apart from acute emergencies. Furthermore, laparotomy delay chemotherapy and might reduce the survival rate.
The sacrococcygeal region may be the origin of germinal tumors, of paragangliomas, and, rarely, of extradural myxopapillary ependymomas (MPE) in the newborn and child. A case is presented of a preterm child with an abdominal tumor, originating from the precoccygeal area, that turned out to be a teratoma with a component of an MPE. The high levels of alpha-fetoprotein in this preterm baby were initially misleadingly interpreted as a tumoral marker. The differential diagnosis and the difficulties in interpreting tumoral markers in infants are discussed.
The purpose of the present study was to determine the effect of surgery on the time length and quality of survival in patients with recurrent glioblastoma multiforme. Two groups were compared; the first included 18 patients who underwent surgery at the time of tumour recurrence. The second group included 36 patients who did not undergo surgery at the time of tumour recurrence. Both groups were matched according to the following criteria: gender, age, Karnofsky Performance Scale (KPS) score, at the time of initial surgery and of tumour recurrence, extent of initial surgery, interval between initial surgery and tumour reccurence. Both groups received conventional treatment after initial surgery. There are no statistically significant differences between the two groups as regards to the previously mentioned criteria. After tumour recurrence, the median survival time was 5 months in the group of patients undergoing a second resection and 2 months in the group of patients not undergoing repeat surgery. The difference was statistically significant on univariate analysis. Moreover, the median length of time spent in an acceptable condition (KPS >/=60) from the time of tumour recurrence was found to be significantly longer in patient who underwent a second resection (4 months) compared with patients who did not undergo repeat surgery (1 month). Even in a relatively favorable subgroup of reoperated patients, the survival benefit although significant was only 3 months. It was impossible to completely match the two groups of patients suggesting that the difference might have been even less. Although symptomatic improvement is modestly achieved by repeat surgery, its transient nature necessitates clear discussion with patient and family on an individual basis.
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UNLABELLED: Complete staging (extensive marrow investigation and meta-iodo benzylguanine (MIBG) scan) is considered as mandatory both at diagnosis and after chemotherapy for assessment of metastases in neuroblastomas. However the correlation between bone marrow invasion and uptake of MIBG at metastatic sites remains unclear. This study investigates whether MIBG alone is sufficiently sensitive to make these procedures redundant. PATIENTS AND METHODS: 20 children over one year of age, with histologically proven metastatic neuroblastoma were studied. Extensive bone marrow assessment and MIBG bone scan performed both at diagnosis and after completion of induction chemotherapy were reviewed. RESULTS: At diagnosis metastases were detected by marrow investigation alone in 2, MIBG alone in 2 and both procedures in 16. After induction chemotherapy metastases were detected by only marrow investigation in 2, by only MIBG in 3, by both procedures in 6 patients, and by none in 9. CONCLUSIONS: Whether marrow investigations and MIBG scan explore the same phenomenon remains unclear. However it appears that marrow disease that is histologically detectable may remain MIBG negative both at diagnosis and after treatment. Both procedures are still justified at time of diagnosis and evaluation of response.