Copper deficiency during parenteral nutrition: a report of four pediatric cases.

The standard of care for patients with cholestasis (direct bilirubin >or=2 mg/dL) while receiving parenteral nutrition (PN) solutions is to reduce or discontinue the copper and manganese. The repercussions of this action have not been studied. Two adult case reports document low serum copper levels associated with clinical symptoms of copper deficiency after the removal of copper from their PN solutions. We now describe the first known series of pediatric patients to develop copper deficiency after copper was removed from their PN solutions.     

Prolonged stenting does not prevent obstruction after TIP repair when the glans was deeply incised.

PURPOSE: To evaluate whether prolonged postoperative stenting may reduce the risk of obstruction of the neourethra after TIP repair with deep glanular incision. METHODS: In a 1-year period 27 patients were operated for penile hypospadias using the TIP technique described by Snodgrass. In contrast to a previous study with 8 - 10 days of postoperative catheter drainage, the indwelling transurethral catheter was kept in place for 12 - 14 days. Deep incision of the urethral plate up to the tip of the glans is the most remarkable surgical detail, resulting in a meatus on top of the glans but in a defect on the dorsal rim of the neomeatus as well. After 3 - 6 months 22 patients were re-investigated during an outpatient visit using a scheme to describe the position of the neomeatus. Uroflowmetry was also performed. Information in three toddlers was gained by phone and 2 patients were lost to follow-up. RESULTS: Two patients returned with significant obstruction including an urethrocutaneous fistula in one. In contrast to the good assessment by parents and compared to the early appearance after catheter removal a change in meatal position was observed in the majority of patients. Only 6 patients presented with an unchanged meatal position whereas in 16 patients the meatus lost its oval or slit-like shape as well as its position on the tip of the glans. However, despite one obstructive meatus in the coronal position 15 patients showed a sufficient size and position of the meatus underneath the tip of the glans. Uroflowmetry revealed reduced peak-flow values (mean: 8.1 ml/s) in some of the 9 patients evaluated. CONCLUSIONS: Our data indicate that prolonged stenting does not give better results in those TIP repairs, in whom the urethral plate was incised across the rim of the neomeatus. The early excellent aspect of the glans after stent removal is often impaired by partial closure of the glans incision with a short-term change in size and position of the meatus. To prevent this, the rim of the meatus during reconstruction should be kept completely epithelialised.     

Use of acute peritoneal dialysis catheters for intraperitoneal chemotherapy instead of the semipermanent/permanent Tenkhoff catheters

Intraperitoneal chemotherapy was administered to 13 patients with peritoneal carcinomatosis, using acute peritoneal dialysis catheters immediately before the administration of the chemotherapy. A total of 59 cycles were administered, with insertion of the corresponding catheter. There were no inflow or outflow problems and no insertion-related complications. With the removal of the catheter after its use, there is no risk of abdominal infections.     

Regular physical activity increases glutathione peroxidase activity in adolescents with Down syndrome.

OBJECTIVE: The present study was designed to determine the influence of a 12-week exercise program on the activity of erythrocyte glutathione peroxidase (GPX) in adolescents with Down syndrome. DESIGN: An interventional study with before-after comparison. SETTING: Sport Medicine School, University of Cadiz (Andalusia, Spain). PATIENTS: Thirty-one male adolescents (16.3+/-1.1) with Down syndrome. None of them suffered acute medical problems at that moment and had not taken part in any physical activity program in the last 6 months. INTERVENTION: A 12-week training program with 3 days per week, consisting of warm up (15 min) followed by a main part (20 to 35 min) at a work intensity of 60% to 75% of peak heart rate (HRmax=194.5-[0.56xage]) and by a cool-down period (10 min). MAIN OUTCOME MEASUREMENT: Erythrocyte activity of GPX. RESULTS: Preexercise and postexercise GPX activity in adolescents with Down syndrome were 24.8+/-3.1 [23.1 to 26.5] U/g hemoglobin and 29.3+/-2.9 [28.1 to 30.5] U/g hemoglobin, respectively. When compared with baseline values it was increased significantly (24.8+/-3.1 vs. 29.3+/-2.9; P=0.011). CONCLUSION: Regular exercise increased significantly GPX activity. Further studies are required to assess the behavior of other antioxidant enzymes to highlight potential benefits of regular exercise in redox metabolism.     

Effect of tacrolimus and partial hepatectomy on transthyretin metabolism in rats

Liver transplantation, which serves as treatment of familial amyloidotic polyneuropathy (FAP), and domino liver transplantation, which utilizes resected livers from patients with FAP for treatment of liver diseases, may induce changes in transthyretin (TTR), a pathogenic FAP-related protein. To evaluate this possibility, we performed a 70% hepatectomy or administered tacrolimus to Dark Agouti (DA) rats for 7 days and then measured changes in liver TTR mRNA levels and changes in serum TTR concentrations. After hepatectomy, TTR mRNA levels decreased by 77%; at day 3, they returned to preoperative levels. Except for slightly elevated serum TTR concentrations 12 h after operation, serum TTR levels remained unchanged. Thus, partial hepatectomy did not influence serum TTR concentrations. After tacrolimus administration, TTR mRNA declined by 56% 12 h after the experiment started; however, after day 3, a rebound phenomenon occurred until day 7. Tacrolimus may facilitate serum TTR degradation, although production of TTR in the liver also increased. This finding -- that TTR, the source of FAP-inducing amyloid, did not increase after transplantation -- may help post-transplantation treatment of patients who have FAP and other liver diseases.     

Onychopathy in a Patient with Fetal Hydantoin Syndrome

Abstract: Hydantoin is an anticonvulsant drug with several side effects. A teralogenic potential has been suggested. The fetal hydantoin syndrome is an entity that consists of a broad range of morphologic and developmental disorders in children born of epileptic mothers exposed to hydantoin during pregnancy. We treated a girl in whom onychopathy was a monosymptomatic or mild form of this syndrome.