Rethinking growth failure in Alagille syndrome: the role of dietary intake and steatorrhea.

Objective: To characterize the energy and nutrient intake, and the degree of fat malabsorption in children with Alagille syndrome (AGS) and to examine their cross-sectional associations with growth and nutritional status.

Methods: Prepubertal children with AGS were evaluated for dietary intake, growth, bone age, and body composition. Children and their families collected a three-day, weighed diet record at home.

Persistent hypercholesterolemia is associated with the development of obesity among girls: the Bogalusa Heart Study.

Background: Obesity is associated with cardiovascular disease (CVD) risk factors. Cross-sectional data suggest that hypercholesterolemia is associated with the development of childhood obesity.

Objective: The objective was to assess age-related changes in relative weight and the association between relative weight and CVD risk factors in hypercholesterolemic and nonhypercholesterolemic children who were nonobese at baseline.

Free and total insulin-like growth factor (IGF)-I levels decline during fasting: relationships with insulin and IGF-binding protein-1.

We have previously demonstrated that IGF-binding protein-1 (IGFBP-1) levels rise steadily during fasting, following an inverse relationship with insulin. The function of the IGFBP-1 rise is unknown, but it has been hypothesized that IGFBP-1 serves as a glucose counterregulatory hormone during fasting and hypoglycemia by binding free IGFs, thus inhibiting IGF interactions with IGF receptors. Our objective in this study was to determine levels of free and total IGFs during fasting together with their interrelationships with simultaneous IGFBP-1, insulin, and glucose levels.

Hospital restructuring and its impact on outcomes: nursing staff regulations are premature.

Objective: Describe restructuring in the organization and delivery of patient care and the effects of nursing structure and processes on selected patient outcomes.

Background: Restructuring has been the dominant cost-reduction strategy in acute care hospitals. Changes occurred without a systematic look at how interventions impacted on the processes and outcomes of care.

Immunologic features of chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome).

Objectives: To characterize immunologic function and clinical characteristics in patients with chromosome 22q11.2 deletion syndrome and determine whether there was significant change over time.

Methods: This study characterized the laboratory and clinical features of the immunodeficiency in a cohort of 195 patients with chromosome 22q11.

Hypoglycaemic effect of aqueous extract of the leaves of Trigonella foenum-graecum in healthy volunteers.

The safety and efficacy of Trigonella foenum-graecum extract was investigated using 20 male volunteers aged 20-30 years. They were randomly treated with either 40 mg/kg aqueous extract powder in 10 mL distilled water or 10 mL distilled water in which coffee simulated the extract. The extract significantly lowered blood glucose level by 13.

In vitro effect of different non-steroidal anti-inflammatory drugs on human polymorphonuclear leukocyte activity measured by luminol-dependent chemiluminescence of the whole blood.

Objective: To define the well-known variability in the effects of non-steroidal anti-inflammatory drugs and to search for predictors of such variability using an in vitro model.

Methods: Polymorphonuclear leukocyte activity was measured by luminol-dependent chemiluminescence of the whole blood using barium sulphate as a stimulator. Blood was taken from 40 apparently healthy volunteers (22 males and 18 females; their age ranged from 20-50 years).

Cocaine withdrawal symptoms and initial urine toxicology results predict treatment attrition in outpatient cocaine dependence treatment.

This study evaluated the ability of cocaine withdrawal symptoms, measured by the Cocaine Selective Severity Assessment (CSSA) and initial urine toxicology results, to predict treatment attrition among 128 cocaine dependent veterans participating in a 4-week day hospital treatment program. The CSSA was administered and a urine toxicology screen was obtained at intake and at the start of the day hospital (about 1 week later). The combination of a positive urine toxicology screen and a high CSSA score at intake predicted failure to complete treatment.

Assessing parental concerns of children with diabetes.

This study assesses levels of parental concern in a group of families who had a young child diagnosed with type 1 diabetes. Despite high levels of concern for hypoglycemia and hyperglycemia, the parents reported that they managed their child's diabetes as recommended by the health care providers. No correlation was found between the concern ratings and level of glycemic control or incidence of severe hypoglycemic events.

Cleft-palate repair by modified Furlow double-opposing Z-plasty: the Children's Hospital of Philadelphia experience.

Although the optimal technique of cleft-palate repair remains controversial, several small series have suggested that superior speech results may be obtained with the Furlow double-opposing Z-plasty. To examine speech outcome in a large series of Furlow palatoplasties performed at a single center, we retrospectively reviewed the records of 390 cleft-palate patients who underwent Furlow palatoplasty at The Children's Hospital of Philadelphia from 1979 to 1992. Speech outcome at 5 years of age or greater was available for 181 nonsyndromic patients and was scored using the Pittsburgh Weighted Values for Speech Symptoms Associated with Velopharyngeal Incompetence.

Palatal length in cleft palate as a predictor of speech outcome.

In an attempt to predict which patients might benefit from primary posterior pharyngeal flaps done at the time of palatal repair, palatal length was assessed before palatal repair and the patient was placed in one of four categories. Patients with longer palates preoperatively had statistically better speech outcomes than patients with shorter palates. Statistical significance was found for most speech parameters.

The long-term effects of non-steroidal anti-inflammatory drugs in osteoarthritis of the knee: a randomized placebo-controlled trial.

Background: Non-steroidal anti-inflammatory drugs (NSAIDs) are widely used to treat osteoarthritis (OA), though their long-term efficacy is uncertain. We report a comparison of the symptomatic responses to therapy with tiaprofenic acid, indomethacin and placebo over 5 yr.

Methods: A parallel-group, randomized, single-blind trial of patients with knee OA recruited 812 patients from 20 centres; 307 patients received tiaprofenic acid (300 mg b.

Longitudinal relationship among growth, nutritional status, and pulmonary function in children with cystic fibrosis: analysis of the Cystic Fibrosis Foundation National CF Patient Registry.

Objective: To determine prospectively the relationship among growth, nutritional status, and pulmonary function over a 4-year period in a large cohort of children with cystic fibrosis (CF).

Study Design: CF Foundation National CF Patient Registry data collected from 1991 to 1995 for 968 children (507 male) aged 5 to 8 years with pancreatic insufficiency and forced expiratory volume in 1 second within 60% to 140% of predicted values (FEV(1)%) were analyzed longitudinally. Variables hypothesized to affect FEV(1)% included age, sex, z scores for height, weight, percent of height-appropriate body weight, and annual number of days hospitalized.

Cleft palate repair at 3 to 7 months of age.

We report the speech outcome in 90 children with complete unilateral cleft lip and palate who underwent soft palate repair either between 3 and 7 months of age (n = 40) or later than 7 months of age (n = 50). In all patients, palatoplasty was performed by one of two experienced surgeons using a modification of the Furlow technique, and speech evaluations were conducted using the Pittsburgh Weighted Values for Speech Symptoms Associated with Velopharyngeal Incompetence by two speech pathologists with high inter-rater reliability. There were no differences between the groups with respect to resonance, nasal air emission, and articulation.

Azathioprine and 6-mercaptopurine for the treatment of perianal Crohn's disease in children.

Numerous adult studies show a 30-65% response rate to azathioprine (AZA) or 6-mercaptopurine (6-MP) for significant perianal Crohn's disease. The aim of this study was to evaluate whether these drugs healed pediatric perianal Crohn's disease. Records of pediatric Crohn's patients were retrospectively reviewed for significant perianal disease treated with AZA or 6-MP for > or =6 months.

Objective evaluation of knee laxity in children.

This study was designed to measure objectively knee laxity in children. Physical examination and the KT 1000 arthrometer were used to test the knee laxity of 150 healthy, uninjured children between 6 and 18 years of age. Data from the knee examinations and the KT 1000 measurements were compared and statistically analyzed to determine the change in knee laxity with age, laxity differences between boys and girls, and the correlation between the KT1000 measurements and subjective tests for laxity described by Carter and Wilkinson.