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1.
Eur J Pediatr ; 168(12): 1505-12, 2009 Dec.
Article in English | MEDLINE | ID: mdl-19301034

ABSTRACT

The aims of this study were to assess the incidence and risk factors of major central venous catheter (CVC)-related complications in a large cohort of children affected by oncological, hematological, or immunological diseases in a 7-year prospective observational study at a single center. Nine hundred fifteen CVCs were inserted in 748 children for a total period of 307,846 CVC-days. Overall, 298 complications were documented with a complication rate of 0.97/1,000 CVC-days: 105 mechanical complications (dislocations 0.30/1,000 CVC-days, ruptures 0.04/1,000 CVC-days), 174 infections (bloodstream infections 0.46/1,000 CVC-days, tunnel infections 0.10/1,000 CVC-days), and 19 thrombosis (0.06/1,000 CVC-days). Significant risk factors were: diagnosis of acute lymphoblastic leukemia (ALL) and age

Subject(s)
Catheterization, Central Venous/adverse effects , Hematologic Diseases/therapy , Infections/epidemiology , Neoplasms/therapy , Upper Extremity Deep Vein Thrombosis/epidemiology , Catheterization, Central Venous/instrumentation , Child , Equipment Design , Female , Hematologic Diseases/complications , Hospitals, Pediatric , Humans , Incidence , Infections/microbiology , Male , Neoplasms/complications , Prospective Studies , Risk Factors , Sepsis/epidemiology
3.
Minerva Pediatr ; 52(4): 205-14, 2000 Apr.
Article in Italian | MEDLINE | ID: mdl-11995204

ABSTRACT

BACKGROUND: The prevalence of seizures in children with acute lymphoblastic leukemia (ALL) varies between 3 and 13% depending on the various studies, whereas it is 1% in the general population aged under 15 years etiopathogenesis and outcome of seizures in children during treatment for ALL. METHODS: A retrospective study was carried out in 204 children with a consecutive diagnosis of ALL, 89 females and 115 males, aged between 5 months and 17 years and 11 months, diagnosed between 15-4-1988 and 15-4-1998, and treated at the Division of Pediatric Oncology and Pediatric Hematology of Turin University using three successive generations of AIEOP protocols 88 (48 cases), 91 (86 cases) and 95 (70 cases). Observation of the patients in the study ended on 30-9-1998. The criteria for eligibility were those stated in the respective protocols. Seizures were classified using the international classification; the diagnosis was made if a doctor, a nurse or a reliable relative witnessed the event with confirmation by the consultant neurologist. RESULTS: Twelve out of 204 (5.8%) patients in this series presented seizures: 2 out of 48 cases using protocol 88 (4.1%), 6 out of 86 cases using protocol 91 (6.9%) and 4 out of 70 cases using protocol 95 (5.7%). None of the patients had critical episodes or other significant neurological pathologies prior to the onset of ALL, nor had they been affected by leukemic meningosis or undergone cranial radiotherapy. When evaluating the possible etiology, the authors noted that, except for one case of febrile convulsion, the seizures in the remaining patients could be attributed to the toxicity of chemotherapy. With regard to the evolution of seizures, only one patient died, whereas the others showed no neurologic sequelae. CONCLUSIONS: The frequency of seizures in children receiving treatment for ALL in the series analysed here is in line with that reported in the literature. Neurotoxicity caused by chemotherapy appears to be the main etiopathogenetic factor.


Subject(s)
Antineoplastic Agents/adverse effects , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Seizures/chemically induced , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Retrospective Studies
4.
Haematologica ; 83(5): 432-7, 1998 May.
Article in English | MEDLINE | ID: mdl-9658728

ABSTRACT

BACKGROUND AND OBJECTIVE: Granulocyte colony-stimulating factor (G-CSF) has been shown to improve the neutropenic status of patients with bone marrow failure. The side effects in prolonged treatment still need to be determined. DESIGN AND METHODS: We have studied the efficacy and the long-term side effects of G-CSF in four patients with Fanconi's anemia and severe neutropenia. RESULTS: Three patients responded with an increase in their absolute neutrophil count; neither improvement in platelet count and hemoglobin concentration nor effect on transfusion requirements was seen. CFU-GM and BFU-E were undetectable before, during and after treatment. Responders showed an important reduction in number and severity of infections, with a marked improvement of clinical status. The fourth patient developed acute myeloid leukemia after 4 weeks of G-CSF treatment. During maintenance, one patient was treated with G-CSF for 18 months, until she received bone marrow transplantation, without presenting side effects. In the second responding patient G-CSF treatment was stopped because of appearance of immature cells in peripheral blood and myeloid blasts in bone marrow. The third responding patient presented immature peripheral myeloid cells during the third year of G-CSF treatment: disappearance of immature cells was observed after G-CSF reduction. In two cases FISH analysis revealed monosomy 7 after G-CSF treatment. INTERPRETATION AND CONCLUSIONS: G-CSF use results in an improvement of clinical status, but long term administration may cause adverse experiences and requires a close hematological monitoring.


Subject(s)
Fanconi Anemia/drug therapy , Granulocyte Colony-Stimulating Factor/therapeutic use , Neutropenia/drug therapy , Adolescent , Child , Fanconi Anemia/complications , Female , Humans , Male , Neutropenia/etiology , Recombinant Proteins
5.
Br J Haematol ; 78(4): 529-34, 1991 Aug.
Article in English | MEDLINE | ID: mdl-1911344

ABSTRACT

We describe a new deletional form of alpha thalassaemia which encompasses the entire alpha-like globin gene cluster in a 15-year-old boy of Southern Italian descent. The deletion removes approximately 31 kb, the 5'-end point is located approximately 4 kb upstream of the xi gene, while the 3'-end point maps between the alpha 1- and theta 1-globin genes. The interaction of this deletion with the common-alpha 3.7 form gives origin to a classical form of haemoglobin (Hb) H disease in the propositus of this study. Deletional forms of xi alpha-thalassaemia are uncommon in the Mediterranean basin; as for other unusual xi alpha-thalassaemia forms, heterozygotes for this mutation may escape detection in population surveys based on zeta and alpha probes.


Subject(s)
Chromosome Deletion , Globins/genetics , Multigene Family/genetics , Thalassemia/genetics , Adolescent , Family , Humans , Immunoblotting , Male
6.
J Inherit Metab Dis ; 12(2): 162-5, 1989.
Article in English | MEDLINE | ID: mdl-2569049

ABSTRACT

Different mutations of the phenylalanine hydroxylase (PAH) gene leading to phenylketonuria (PKU) have been described associated with specific haplotypes in several European countries. In order to investigate the distribution of DNA haplotypes in Italy, restriction fragment length polymorphism (RFLP) analysis of the PAH gene was performed in nine Italian PKU patients from eight unrelated families, and in the available relatives. The analysis of eight polymorphic sites revealed haplotypes 1 and 6 in association with PKU. This pattern appears to differ from those reported for other European populations. The majority of the 14 PKU subjects studied showed compound heterozygosity for different haplotypes, as observed for other European series. RFLP analysis at the PAH locus allowed us to offer the possibility of prenatal diagnosis to six of the studied families. One prenatal diagnosis was performed and a normal fetus was diagnosed.


Subject(s)
Phenylalanine Hydroxylase/genetics , Phenylketonurias/genetics , Polymorphism, Genetic , Polymorphism, Restriction Fragment Length , Alleles , Female , Gene Frequency , Haplotypes , Heterozygote , Humans , Italy , Phenylketonurias/diagnosis , Pregnancy , Prenatal Diagnosis
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