High-density lipoprotein subclasses and esterification rate of cholesterol in children: effect of gender and age

Since the development of coronary heart disease (CAD) is affected by a specific pattern of plasma high density lipoprotein (HDL) effects it may be useful to know whether this occurs already in childhood. In this study we evaluated particle size distribution of HDL by gradient gel electrophoresis and the determination of cholesterol esterification rate (FER_HDL) in plasma depleted of apo B lipoproteins in 221 children (108 boys and 113 girls) aged 4 months to 20 years. Total plasma- (TC), low-density lipoprotein- (LDL-C) and HDL- (HDL-C) cholesterol, HDL unesterified cholesterol (HDL-UC) and plasma triglycerides (TG) were also measured. There were no significant gender and age differences with respect to the plasma TC, LDL-TC and TG but concentration of HDL-TC increased with age. Post-pubertal girls had significantly higher relative concentrations of HDL_2b compared to boys (30.4% vs 17.2%), while HDL_3b,c was lower in post-pubertal girls (8.7% vs. 16.5 %). FER_HDL correlated inversely with HDL_2b and positively with HDL_3b,c particles and was significantly higher in boys of the post-pubertal group compared to girls (16.9%/h vs 12.5%/h). While in girls there was a positive correlation between age and HDL-C, HDL-UC and the relative concentration of HDL_2b no significant correlation were observed in boys. In girls the increase in TC showed a significant correlation with a simultaneous increase in HDL-C, HDL-UC and HDL_2b. In boys TC correlated significantly with changes in TG only. When HDL_2b and HDL_3b,c cholesterol levels are calculated from HDL-C concentration and per cent distribution the differences between males and females are further emphasized. These data indicate that HDL particle size distribution is age- and gender-dependent.     

Hypoglycémie hyperinsulinémique persistante du nouveau-né et du nourrisson

Persistent hyperinsulinemic hypoglycaemia of infancy (PHHI) is the most frequent cause of hypoglycaemia in infancy. Clinical presentation is heterogeneous, with variable onset of hypoglycaemia and response to diazoxide, and presence of sporadic or familial forms. Underlying histopathological lesions can be focal or diffuse. Focal lesions are characterised by focal hyperplasia of pancreatic islet-like cells, whereas diffuse lesions implicate the whole pancreas. The distinction between the two forms is important because surgical treatment and genetic counselling are radically different. Focal lesions correspond to somatic defects which are totally cured by limited pancreatic resection, whereas diffuse lesions require a subtotal pancreatectomy exposing to high risk of diabetes mellitus. Diffuse lesions are due to functional abnormalities involving several genes and different transmission forms. Recessively inherited PHHI have been attributed to homozygote mutations for the beta-cell sulfonylurea receptor (SUR1) or the inward-rectifying potassium-channel (Kir6.2) genes. Dominantly inherited PHHI can implicate the glucokinase gene, particularly when PHHI is associated with diabetes, the glutamate dehydrogenase gene when hyperammonaemia is associated, or another locus.     

Comparison of point-of-care activated clotting time systems utilized in a single pediatric institution

This study compares four different activated clotting time (ACT) point-of-care (POC) testing systems used at our institution for the management of patients undergoing heparin therapy. We evaluated these systems under identical conditions to determine their accuracy, reproducibility, ease of use, and cost. Two separate testing stations containing four ACT systems were used. The testing order was randomized for every sample and performed by two trained individuals. Samples of fresh heparinized whole blood were taken at regular intervals and distributed to each station. Each operator tested 50 samples, totaling 400 ACT tests. The ACT value was significantly affected by the type of machine used at both stations 1 and 2 (p < .001). Compared with all systems, the Medtronic ACT Plus Automated Coagulation Timer System (ACT Plus) resulted in the most consistent ACT values (median = 171, Interquartile Range (IQR): 169-175) and least variability (172.17 +/- 5.24). The Hemochron Signature Elite Whole Blood Microcoagulation System had the most variability (221.10 +/- 14.78) and yielded consistently higher ACT values (median = 220, IQR: 210-229.5) compared with other systems. The ACT values reported by the i-STAT Handheld and Test Cartridge Blood Analysis System (153.30 +/- 7.87) were consistently lower (median = 154, IQR: 147-161) in comparison to the ACT Plus and Medtronic HMS Plus Hemostasis Management System (180.60 +/- 7.60, median = 181, IQR: 175-186). There was no statistical difference in results between the two testing sites (p > .05) or the operators (p > .05). The significant finding of this study was the affect each system has on the ACT value. This investigation demonstrates the variability that exists among different ACT monitoring systems at our institution. The discrepant variation in ACT values that exists with the Hemochron system questions the reliability of its use in the management of patients undergoing heparin therapy.     

Pancreas islet transplantation in patients with type 1 diabetes mellitus after kidney transplantation

Diabetic patients with end-stage renal disease have a high mortality rate. A combined kidney-pancreas transplant is associated with greater life expectancy. Pancreas islet transplantation is an alternative involving a lower degree of morbidity. We present two patients, of 41 and 37 years of age, with a long history of diabetes mellitus (C-peptide negative), both with a previous kidney transplant, who had been treated with 22 and 28 U of insulin/d, respectively. Both patients had frequent episodes of unawareness hypoglycemia. Pancreatic islets were infused to a total of 7809 and 19,180 IE/kg, respectively. Basal posttransplant C peptide levels were 2.9 and 1.3 ng/mL. After the implant, one patient required occasional doses of insulin, and the other patient more than 50% reduced dose. After the first implant neither patient had any episodes of unawareness hypoglycemia. HbA1c at 4 months were 6.2% and 6.9%. There were no transplant-related complications.     

Results of gastric bypass plus resection of the distal excluded gastric segment in patients with morbid obesity

Surgical treatment is the procedure of choice for morbidly obese patients. Gastric bypass with a long limb Roux-en-Y anastomosis is the "gold standard" technique for these patients. We sought to determine the early and late results of open gastric bypass with resection of the distal excluded stomach in patients with morbid obesity. We included in this prospective study 400 patients who were seen from September 1999 through August 2003 (311 women and 89 men; mean age, 38.5 years). The mean body mass index of the patients was 46 kg/m2. All underwent 95% distal gastrectomy, with resection of the bypassed stomach, leaving a small gastric pouch of 15 to 20 ml. An end-to-side gastrojejunostomy was performed with circular stapler No. 25. The length of the Roux-en-Y loop was 125 to 150 cm. In all patients, a biopsy was taken from the liver and routine cholecystectomy was performed. Follow-up was as long as 36 months. A barium study was performed in all patients at 5 days after surgery. Mortality and postoperative morbidity rates were 0.5% and 4.75%, respectively, mainly due to anastomotic leak in 10 patients (2.5%). Hospital length of stay was 7 days for 95% of the patients. Follow-up data for longer than 12 months were available in 184 patients. There was excess body weight loss of 70% at 24 and 36 months, and there was an inverse correlation among preoperative body mass index and the loss of weight. Anemia was present in 10%, and incisional hernia was present in 10.2%. At 1 year after surgery, the BAROS index demonstrated very good or excellent index in 96.6% of the patients. Gastric bypass with resection of the distal excluded segment has results very similar to those of gastric bypass alone but eliminates the potential risks of gastric bypass such as anastomotic ulcer, gastrogastric fistula, postoperative bleeding due to peptic ulcer and gastritis, and the eventual future development of gastric cancer. It is also possible to perform via laparoscopy, as we started to do recently.     

Chronic kidney disease in renal transplant recipients

The aim of the present study was to investigate the utility in renal transplant patients of the guidelines for the diagnosis and classification of chronic kidney disease (CKD) based on the estimated glomerular filtration rate (GFR) elaborated by the Kidney Disease Outcomes Quality Initiative (K/DOQI) of the National Kidney Foundation. PATIENTS AND METHODS: Four hundred forty-seven cadaveric kidney transplants performed between 1980 and 1994 with graft function at 12 months were included in the study. The GFR was calculated according to the MDRD equation. RESULTS: The mean GFR at 12 months was 54.5 +/- 20.3 mL/min/1.73 m(2): 23 patients (5.1%) had a GFR > or =90 mL/min/1.73 m(2); 136 patients (30.6%), 60-89; 246 (54.7%), 30-59; 35 patients (7.8%), 15-29; and 7 patients (1.6%), GFR <15. Similar distribution of CKD stages was observed at 5 and 10 years. Unadjusted graft survival at 10 years was better among patients with a higher GFR at 12 months: 87% in patients with GFR >90 mL/min/1.73 m(2); 83% of GFR 60-89 mL/min/1.73 m(2); 63%, GFR 30-59 mL/min/1.73 m(2); and 23%, GFR <30 mL/min/1.73 m(2) (P < .001). The association between GFR and graft survival persisted when adjusted by the age and gender of the recipients and donors, time on dialysis, body mass index, immunosuppression, delayed graft function, rejection, and HLA mismatches. The prevalence of complications, such as anemia, hypertension, dyslipidemias, and number of drugs increased as GFR declined. CONCLUSIONS: More than 60% of recipients presented chronic kidney disease. GFR was a predictive factor for graft survival at 10 years. The classification of renal transplant patients by CKD stages may help to identify patients with increased risk of graft loss and also to design strategies to improve outcomes.     

Use of risk stratification to target therapies in patients with recent onset arthritis; design of a prospective randomized multicenter controlled trial

Background  

Early and intensive treatment is important to inducing remission and preventing joint damage in patients with rheumatoid arthritis. While intensive combination therapy (Disease Modifying Anti-rheumatic Drugs and/or biologicals) is the most effective, rheumatologists in daily clinical practice prefer to start with monotherapy methotrexate and bridging corticosteroids. Intensive treatment should be started as soon as the first symptoms manifest, but at this early stage, ACR criteria may not be fulfilled, and there is a danger of over-treatment. We will therefore determine which induction therapy is most effective in the very early stage of persistent arthritis. To overcome over-treatment and under-treatment, the intensity of induction therapy will be based on a prediction model that predicts patients' propensity for persistent arthritis.