[Gas chromatographic study of fecal hydroxyacids in infants with steatorrhea due to cystic fibrosis (with and without pancreatin treatment) and to celiac disease].
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Celiac Disease↗
Biomedical subjects
A Donfrancesco
Publications and source records attributed to A Donfrancesco.
[Reye's syndrome: pathogenetic problems (author's transl)].
The authors describe six subjects with Reye's syndrome. All subjects died nevertheless the treatment (exchange-transfusions infusions of citrulline and ornithine). The autoptical studies showed cerebral oedema and fatty degeneration of the liver. Hepatic and seric OTC activity was measured in three patients: enzyme activity was virtually absent in one patient and normal in the other two. Instead in one patient was found partial CPS deficiency. However, Reye's syndrome is not only correlated with enzymatic deficiency of urea's cycle but sometimes also with toxic and metabolic causes.
Child↗
[Relapse modalities in medulloblastoma].
We evaluated the modality of relapse and progression of medulloblastoma in 45 patients observed between 1981-1991. Children aged over 2 years were treated with surgery and postoperative radio-chemotherapy; among 12 children younger than 2 years, 8 were treated with surgery and chemotherapy and 4 with postoperative irradiation of remaining tumor. Cerebrospinal fluid shunting system was placed in 32 patients (71.1%). Patients were organized into three groups: group I (25 cases) = total tumor removal; group II (11 cases) = subtotal tumor removal; group III (9 cases) = partial tumor removal. 22 children died (48.8%: 10 of group I; 7 of group II; 5 of group III) at variable time interval from the operation during the following period. Among 23 alive patients, 3 are surviving with recurrence and progression of disease (all of group I), 20 are disease-free (44.4%: 12 of group I; 4 of group II; 4 of group III). Average postoperative follow-up period: 6 years for group I/II and 2 years for group III. Even if disease-free children are those treated by total-subtotal surgical removal (while local relapse occurs principally in patients treated by partial surgical resection: 44.4% of local relapse vs 12% of group I and 27% of group II), extensive surgery does not exclude possibility of metastatic localization in cerebrospinal axis, that occurs in 1/5-1/6 of all the patients without differences in each group. CSF shunting system did not cause extraneural metastases in our patients. We obtained long term survival rates or apparent recovery in almost 50% of cases.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Peripheral blood stem cells in children with solid tumors. Part II. Immunocytologic detection of tumor cells in bone marrow and peripheral blood stem cell harvests.
Simultaneous comparative assessment of tumor contamination in bone marrow aspirates and peripheral blood stem cell collections using immunocytochemical techniques was done in 6 children with neuroectodermal tumors. In 3 neuroblastoma patients tumor cells were detected in 5 of 16 marrow samples but not in peripheral blood stem cells. Clonogenic tumor cell reinfusion in the autologous support setting may be avoided in neuroblastoma patients by using peripheral blood stem cells.
Adolescent↗
D-CECaT as preoperative chemotherapy for unresectable neuroblastoma in children over one year of age.
The role of intensive pre- and postoperative chemotherapy in unresectable nonmetastatic neuroblastoma is still controversial. A preoperative regimen that included deferoxamine, cyclophosphamide, etoposide, carboplatin and thiotepa (D-CECaT) was evaluated in 10 children over one year of age at diagnosis, and this was followed by surgery and postoperative chemotherapy. After four courses of D-CECaT, the response rate was 9/10 with 3 complete responses, 6 partial responses and 1 minor response. Severe but transitory myelosuppression was the major toxic effect. Complete remission by combined D-CECaT chemotherapy and surgery was obtained in 9/10 patients, while 1 case achieved complete remission only with postoperative chemotherapy. All children are disease-free with a median follow-up of 30.5 months (range: 1+ to 50+). This intensive treatment was effective in both standard- and high-risk unresectable NB. However, whether a less intensive approach and fewer courses can also give similar results in standard-risk cases warrants further study.
Antineoplastic Combined Chemotherapy Protocols↗
Shortened time to recovery from chemotherapy induced neutropenia in pediatric patients with high dose combined cytokines.
Granulocyte colony stimulating factor (G-CSF) and granulocyte macrophage colony stimulating factor (GM-CSF) are cytokines which have been extensively administered as monotherapy to patients with a variety of hematopoietic disorders at dosages of 5 mcg/kg/day. Because their spectrum of activity is both singular and simultaneously overlapping, we postulated that combined therapy would be more advantageous than monotherapy. Since 1992 we have carried out a study of G-CSF and GM-CSF as monotherapy or in combination in pediatric patients with solid tumors following chemotherapy induced nadirs of 0-800 WBC/mm3. When combined, the cytokines were given twice per day at 2.5 or 5.0 mcg/kg. For the monotherapy groups, either cytokine at 5 mcg/kg or 10 mcg/kg was given once daily. The mean time to recovery from neutropenia nadir ranged from 6.6-8.2 days in patients receiving a total of 10 mcg/kg/day compared to 10.4-10.6 days in patients treated with 5 mcg/kg/day. Side effects were ephemeral eosinophilia. The dosage of 10 mcg/kg/day appears to be a better dosage for pediatric patients with a slight advantage in the combined twice a day schedule (6.6 days).
Adolescent↗