ABSTRACT
Thirty-two children and adolescents received their renal transplant at the Montefiore Medical Center, in New York, between October 1996 and May 2000. Twenty-four patients received basiliximab, in addition to tacrolimus and steroids (basiliximab group). The remaining eight patients received only tacrolimus and steroids (non-basiliximab group). The 1-year patient survival rate was 100% in both groups. The 1-year graft survival rate was 87.5% for the basiliximab group and 75% for the non-basiliximab group (P=0.45). The rates of acute rejection in the basiliximab and non-basiliximab groups were 26% and 43%, respectively (P=0.36). However, in recipients with Subject(s)
Antibodies, Monoclonal/therapeutic use
, Immunosuppressive Agents/therapeutic use
, Recombinant Fusion Proteins
, Adolescent
, Antibodies, Monoclonal/adverse effects
, Basiliximab
, Child
, Child, Preschool
, Diabetes Mellitus/etiology
, Female
, Humans
, Kidney Transplantation/adverse effects
, Kidney Transplantation/mortality
, Lymphoproliferative Disorders/etiology
, Male
, Survival Rate
ABSTRACT
We report the identification of a novel Wilms tumor suppressor gene mutation in a 5-month-old girl who presented with unilateral Wilms tumor (WT) and renal diffuse mesangial sclerosis typical of Denys-Drash syndrome (DDS). The patient did not have ambiguous genitalia and the karyotype (by amniocentesis) was 46, XX. A de novo constitutional heterozygous mutation in WT1 gene exon 9 coding for the third zinc-finger (1163G-->A, C388Y) was identified. This mutation affects a cysteine residue involved in the coordination of the zinc atom, confirming the importance of these residues in the biological function of WT1 protein.
