Severe intrarenal fibrosis, infundibular stenosis, renal cysts, and persistent perilobar nephrogenic rests in a patient with Beckwith-Wiedemann syndrome 27 years after diffuse nephroblastomatosis and Wilms tumor: natural progression or a consequence of treatment?

A27-year-old woman presented with back and abdominal pain. She was diagnosed in infancy with Beckwith-Wiedemann syndrome and bilateral multifocal perilobar nephrogenic rests that progressed to diffuse nephroblastomatosis with neoplastic nephroblastomatous rests at 14 months of age and subsequently to a right Wilms tumor at 5 years of age. Computed tomography of the abdomen during the current admission showed multiple obstructed calices.

Bioelectrical impedance analysis of the body composition of children and adolescents with sickle cell disease.

Objectives: To examine the body composition of children and adolescents with sickle cell disease (SCD) using bioelectrical impedance analysis and to determine if the impedance parameters resistance, reactance, and phase angle are able to distinguish between subjects with SCD and age- and gender matched controls.

Study Design: Total body resistance and reactance were obtained for a total of 53 subjects with SCD (27 male and 26 female) between 10 and 18 years of age and 49 control subjects (23 male and 26 female). The fat-free mass, body cell mass, phase angle, and capacitance were also determined.