A cross-species approach to identify transcriptional regulators exemplified for Dnajc22 and Hnf4a.

There is an enormous need to make better use of the ever increasing wealth of publicly available genomic information and to utilize the tremendous progress in computational approaches in the life sciences. Transcriptional regulation of protein-coding genes is a major mechanism of controlling cellular functions. However, the myriad of transcription factors potentially controlling transcription of any given gene makes it often difficult to quickly identify the biological relevant transcription factors. Here, we report on the identification of Hnf4a as a major transcription factor of the so far unstudied DnaJ heat shock protein family (Hsp40) member C22 (Dnajc22). We propose an approach utilizing recent advances in computational biology and the wealth of publicly available genomic information guiding the identification of potential transcription factor candidates together with wet-lab experiments validating computational models. More specifically, the combined use of co-expression analyses based on self-organizing maps with sequence-based transcription factor binding prediction led to the identification of Hnf4a as the potential transcriptional regulator for Dnajc22 which was further corroborated using publicly available datasets on Hnf4a. Following this procedure, we determined its functional binding site in the murine Dnajc22 locus using ChIP-qPCR and luciferase assays and verified this regulatory loop in fruitfly, zebrafish, and humans.

Summer camps for children and adolescents with kidney disease.

Summer camps, sports camps, and residential camps are readily available to children and adolescents across the country. However, children and adolescents with end stage renal disease (ESRD) may not be able to participate in summer camp experiences because of specialized medical needs (e.g., dialysis or immunosuppressive medications) and concerns about abilities to keep up with camp activities. With enhancements in pediatric nephrology care in the past 10 years, patients can be expected to attend school full time and participate in peer activities. In addition, attendance at summer camps becomes a possibility for these children, particularly camps aimed at children with ESRD. Twenty pediatric nephrology centers in North America were surveyed about their participation in summer camp programs. This article reviews these and attempts to elucidate the values of summer camp programs for pediatric ESRD patients.

Continuous venovenous hemofiltration with and without dialysis in pediatric patients.

Peritoneal dialysis (PD) is often the preferred modality in dialyzing the pediatric patient in acute renal failure. However, PD may be contraindicated in the presence of the acute surgical abdomen, respiratory compromise, or diaphragmatic disruption. The child's size and cardiovascular instability may also render hemodialysis undesirable. The use of continuous arteriovenous hemofiltration (CAVH) has been an option for the acutely ill child but requires arterial and venous access as well as adequate blood pressure to drive the CAVH circuit. Another option is continuous venovenous hemofiltration (CVVH), which obviates the need for arterial access and provides blood flow via an external pump. This article presents a retrospective of 20 acutely ill pediatric patients who received continuous venovenous hemofiltration with and without dialysis (CVVH/D) during the period covering Fall 1992 through Fall 1993 at Children's Hospital in Seattle. The children ranged in age from 1 day to 12 years (mean age 4 years) and weights ranged from 1.7 kg to 76 kg (mean 15.8 kg). Seventeen of the 20 patients were started on CVVH/D due to hemodynamic instability, 1 for PD complications, and 2 for metabolic disorders. Fluid and solute removal were achieved efficiently and metabolic imbalances were easily corrected. Patients received 1-25 days (mean 7.7 days) of CVVH/D.

Peritoneal equilibration test curves and adequacy of dialysis in children on automated peritoneal dialysis.

Peritoneal equilibration tests (PETs) and adequacy studies have been performed to guide dialysis prescriptions in adult continuous ambulatory peritoneal dialysis patients, although few studies have been reported in pediatric patients on nightly cycling peritoneal dialysis. We performed 49 PETs in 28 children on automated peritoneal dialysis (mean age, 8.9 years; age range, 0.2 to 19.8 years; mean time on dialysis, 14.1 months) using Dianeal 2.5% dialysate (Baxter Healthcare Corp, McGaw Park, IL) inflow volumes of 1,200 mL/m2 (approximately 40 mL/kg), and standard technique. Mean 4-hour dialysate/plasma creatinine was 0.73 +/- 0.12 (range, 0.45 to 1.03). Mean 4-hour dialysate glucose/initial dialysate glucose was 0.28 +/- 0.10 (range, 0.06 to 0.50). Eighty percent of these PETs fell into the high average/high solute transport categories proposed by Twardowski, based on adult PETs. Twenty-four-hour dialysate collections were analyzed in 28 patients for protein loss and glucose absorption, as well as for calculation of weekly Kt/V. Mean weekly Kt/V was 1.94 +/- 0.94 (range, 0.63 to 5.10). Mean dialytic creatinine clearance calculated from the 4-hour dwell was 5.6 +/- 1.0 mL/min/1.73 m2 (56 L/wk/1.73 m2). Mean daily protein loss was 0.20 +/- 0.13 g/kg/d (range, 0.2 to 9.1 g/d) and glucose absorption was 3.49 +/- 2.24 g/kg/d (range, 17 to 196 g/d). Our results show higher solute diffusion rates in children compared with the published adult PET results (P = 0.0013 and P < 0.0001 for 4-hour creatinine and glucose results, respectively), with trends toward more rapid solute transport in younger compared with older children.(ABSTRACT TRUNCATED AT 250 WORDS)

Pediatric case study: hemolytic uremic syndrome.

The nursing interventions necessary to care for this critically ill child were professionally challenging. Meeting the psychosocial, educational, and supportive needs of the family during this period of uncertainty, as well as, providing the dialysis therapy and support for this fragile child demonstrated the unique contributions of professional nursing. All outcomes were met, but the patient's extremely poor myocardial function made dialysis treatments and volume control difficult. The use of CVVHD was instrumental to providing dialysis in a safe, effective manner. The nursing care for S.B.K. and her family was physically and emotionally draining, but seeing her neurological status return was the great reward for all of our efforts.

Benefits of a mainstreamed summer camp experience for teens with ESRD.

This is a report on a pilot project integrating children with end stage renal disease (ESRD) with well children for a summer camp experience. As the teen with ESRD prepares to enter the work force and college, he/she will have to adapt to a variety of situations that will not adapt to his/her unique medical condition. These issues motivated a pilot project in which 9 ESRD children were mainstreamed into a 2 week, YMCA summer camp experience. Pre and post questionnaires were developed and distributed to the camper, family, and the cabin counselor along with interviews to assess the value of the experience. All the children left camp more independent and knowledgeable about their self-care. The results of this pilot project indicate that children with ESRD can adapt to their environment and increase independence, self-care and self-esteem through supervised mainstreamed experiences.