Localization and therapy of urinary tract infections of childhood.

One hundred four patients with 124 episodes of urinary tract infection were studied. Serum C-reactive protein (CRP) was determined on diagnosis of each patient. Children with a CRP equal to or greater than 30 micrograms/ml (CRP-pos) differed significantly from those with values less than 30 micrograms/ml (CRP-neg) in age, clinical presentation, K type of Escherichia coli causing disease, frequency or radiographic abnormalities, and presence of antibody coating of bacteria in the urinary sediment. E. coli K1 strains caused disease significantly more often in CRP-pos than in CRP-neg patients, and children with K1 infections were younger than those with non-K1 infections. The antibody-coated bacteria test was neither sensitive nor specific for localization of infection in pediatric patients. Determination of K1 antibody concentrations in serum and urine of E. coli K1-infected children provided data supporting the measurement of CRP as one means of localizing urinary tract infections. Patients with CRP-neg infections were treated as successfully with four days of antimicrobial therapy as with ten days.

Treatment of children with homozygous familial hypercholesterolemia: safety and efficacy of low-density lipoprotein apheresis.

We evaluated the safety and efficacy of dextran sulfate low-density lipoprotein (LDL) apheresis in the treatment of three children (aged 6, 7, and 10 years) with severe familial homozygous hypercholesterolemia and undetectable LDL receptor activity. A total of 35 double plasma volume procedures were performed. The ranges of the mean decreases of the three patients in plasma lipid concentrations after LDL apheresis (p less than 0.0001) were as follows: total cholesterol, 76% to 79%; LDL-cholesterol, 78% to 81%; very low density lipoprotein cholesterol, 69% to 75%; high-density lipoprotein cholesterol, 27% to 40%; and triglycerides, 34% to 68%. There were statistically significant but clinically and biologically irrelevant changes in hematologic indexes, serum chemistry values, immunoglobulin levels, complement activity, and plasma concentrations of fat-soluble vitamins. Simple correlation analysis of the variables affecting total cholesterol removal showed significant correlation coefficients (r values) for preapheresis total cholesterol values (r = 0.70; p less than 0.01) and preapheresis LDL-cholesterol values (r = 0.61; p less than 0.01). A multiple regression model explained 82% of the variance based on the preapheresis cholesterol concentration, volume of whole blood processed, and the serum albumin concentration. Side effects of the LDL-apheresis treatments were rare and included abdominal cramping and urticaria. Two procedures were aborted because of intravenous access problems in the younger children. This study confirms that LDL apheresis using a dextran sulfate affinity column is efficacious in rapidly lowering total and LDL-cholesterol concentrations. Furthermore, the procedure is safe and well tolerated by children as young as 6 years of age. This treatment may prevent the progression of atherosclerosis in children with homozygous familial hypercholesterolemia and may therefore avert early death.

Low-density lipoprotein apheresis as long-term treatment for children with homozygous familial hypercholesterolemia.

OBJECTIVE: To determine the safety and efficacy of long-term dextran sulfate-affinity column low-density lipoprotein (LDL) apheresis for the treatment of children with receptor-negative homozygous familial hypercholesterolemia (HFH). STUDY DESIGN: Two children with HFH (pretreatment cholesterol levels 22.1 to 24.7 mmol/L (ranges 850 to 950 mg/dl) began LDL apheresis treatments at ages 7 and 10 years, respectively. The LDL apheresis treatment interval was generally either 7 or 14 days; for the last 2 years of the study the treatment interval was 7 days. The patients had 167 and 188 LDL apheresis procedures during 64 and 70 months, respectively. RESULTS: Individual procedures decreased total blood cholesterol levels by 63% to 68%. When the treatment interval was 7 days, the patients' time-averaged mean total cholesterol levels decreased to 7.3 +/- 0.65 mmol/L (280 +/- 25 mg/dl) and 6.4 +/- 0.55 mmol/L (247 +/- 22 mg/dl), respectively. Both children remained clinically well with normal growth and development. There was significant regression of xanthomas in both patients. The older patient required heart surgery for preexisting aortic stenosis and coronary ostial stenosis, but neither patient had progression of hypercholesterolemia-related cardiovascular disease. With the exception of iron (deficiency in patient 1), there was no evidence of depletion of serum components. Adverse reactions to LDL apheresis were rare and never severe. CONCLUSIONS: Dextran sulfate-affinity column LDL apheresis is effective long-term treatment for children with receptor-negative HFH.