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Biomedical subjects

A Gassas

Publications and source records attributed to A Gassas.

5 recordsLinked to original sources

Comparative outcome of hematopoietic stem cell transplantation for pediatric acute lymphoblastic leukemia following cyclophosphamide and total body irradiation or VP16 and total body irradiation conditioning regimens.

To compare the outcome of hematopoietic stem cell transplantation (HSCT) in pediatric acute lymphoblastic leukemia (ALL) conditioned with two different regimens: (1) single dose of VP16 (60 mg/kg over 4 h) and total body irradiation (TBI; 1200 cGy, in six fractions) or (2) Cyclophosphamide 50 mg/kg over 1 h daily for 4 days followed by the same dose of TBI. One hundred and seven children with ALL received fully matched HSCT from 1990 to 2003 in the Hospital for Sick Children, Toronto. All received cyclosporin A and a short course of methotrexate for graft-versus-host disease (GVHD) prophylaxis. The VP16 group, there were 36 matched related donor transplants (MRD) and 26 matched unrelated donor transplants (MUD), and in the cyclophosphamide group there were 23 MRD and 22 MUD transplants. Neutrophil engraftment occurred at a median of 18 and 17 days for the VP16/TBI and the CY/TBI groups, respectively. The 3 year event-free survival and overall survival were 47 +/- 7 and 55 +/- 7% for those receiving VP16/TBI, and 51 +/- 8 and 53 +/- 8% for the CY/TBI group. There were no significant differences in the prevalence of acute or chronic GVHD and transplant-related mortality between the two groups. Both VP16/FTBI and CY/FTBI regimen are equally effective regimens.

Adolescent↗

Risk of ventriculoperitoneal shunt infections due to gastrostomy feeding tube insertion in pediatric patients with brain tumors.

OBJECTIVE: To determine the risk of ventriculoperitoneal (VP) shunt infections after percutaneous retrograde gastrostomy feeding tube (GT) placement in children with brain tumors. PATIENTS AND METHODS: All children (age 0-18 years) with primary brain tumors diagnosed and treated at the Hospital for Sick Children, Toronto, Canada, were subjected to a retrospective analysis. Two groups were identified: the study group included children with a VP shunt and a GT; the control group included children with VP shunts only. Each study patient was matched with 2 controls to compare the rate of infections (cohort comparative study). RESULTS: There were 1,167 children diagnosed and treated with primary brain tumors during the study period (1988-2003); 174 (15%) had a VP shunt and 23 (2%) children had both, a VP shunt and a GT. In the study group (n=17), GTs were inserted at a median time of 80 days (range 6-204 days) after VP shunts. VP shunt infection rate was 23.5% (4/17) compared to 8.8% (3/34) in the control group (OR 3.18; 95% CI 0.622-16.54, p=0.16). Three (75%) of the infection episodes in the study group presented with an ascending VP shunt infection directly related to the GT insertion or manipulation in the first 6 weeks. These GTs were inserted at 13, 47 and 49 days after VP shunt insertion. CONCLUSION: Placement of percutaneous retrograde GTs, in the acute phase, in children with brain tumors and VP shunts may increase the risk of ascending meningitis especially if there are early GT-related complications.

Adolescent↗

Life-threatening pulmonary hemorrhages post bone marrow transplantation in Hurler syndrome. Report of three cases and review of the literature.

Hurler syndrome (MPS-IH) is an autosomal recessive mucopolysaccharide storage disorder caused by deficiency of lysosomal alpha-L-iduronidase (IDU) enzyme activity. This results in accumulation of heparan sulfate and dermatan sulfate substances. Untreated children develop progressive developmental deterioration and multisystem morbidity with a median survival of 5 years. Allogeneic bone marrow transplantation (BMT) is the only long-lasting treatment that ameliorates or halts the aggressive course of the disease. Pulmonary hemorrhage (PH) is an unusual complication of BMT and has not been previously reported in MPS-IH post-BMT. We report three children with MPS-IH with life-threatening PH around the time of engraftment. All needed intensive-care support and one child developed recurrent PH that required prolonged ventilation.

Bone Marrow Transplantation↗

The effect of prelung transplant clinical status on post-transplant survival of children with cystic fibrosis.

The aim of this study was to determine whether transplanting paediatric cystic fibrosis (CF) patients later in the course of their disease was detrimental to their post-transplant survival. Data was collected from 51 children with CF undergoing lung or heart-lung transplantation May 1988-March 1999. The following risk factors were tested by Cox proportional hazards modelling: age at transplant; sex; donor/recipient sex mismatch; donor/recipient cytomegalovirus (CMV) mismatch; cold and warm graft ischaemic times; and donor age. Pretransplant forced expiratory volume in one second (FEV1), minimum oxygen saturation obtained during 12 min walk (Sa,O2min), and a survival probability score (SP) calculated from FEV1, age adjusted resting heart rate, age, sex, blood haemoglobin (Hb), and serum albumin were then added to the model. None of the risk factors were significantly correlated with death during the study period. No evidence that clinical status prior to transplant has any effect upon the post-transplant survival of children with cystic fibrosis was found.

Adolescent↗