Catheter-related infections associated with home parenteral nutrition and predictive factors for the need for catheter removal in their treatment.

The inpatient and outpatient records were reviewed for 527 patients, including 138 children, who were discharged on home total parenteral nutrition and followed by the University of California, Los Angeles, home total parenteral nutrition service for a minimum of 1 week between April 1973 and October 1991. The total follow-up time was 1154 patient years; the median follow-up time was 206 days (range 7 to 6344 days). Thirty-six patients were followed for more than 10 years (median 12.

Mucosal bridge formation in the esophagus caused by injury from a nasoenteric feeding tube.

We report the first case of an esophageal mucosal bridge that was caused by a nasoenteric feeding tube or its placement. The mucosal bridge was diagnosed by esophagoscopy and was nearly resolved 3 weeks later after removal of the feeding tube and treatment with histamine antagonists. Although uncommon, esophageal injury may occur in relation to the use of small-bore nasoenteric feeding tubes and great care should be taken during tube placement and use.

Selenium renal homeostasis is impaired in patients receiving long-term total parenteral nutrition.

Selenium deficiency has been reported previously in patients receiving long-term total parenteral nutrition (TPN) without selenium supplementation in their solutions. The recommended dietary allowance for selenium is 0.87 microgram/kg, of which 80% is absorbed.

Choline pharmacokinetics during intermittent intravenous choline infusion in human subjects.

A study of choline pharmacokinetics was undertaken in four patients receiving long-term total parenteral nutrition. On consecutive days, 7, 14, 28, and 56 mmol choline chloride were intravenously infused over a 12-hour period in each subject. The choline concentration was determined in plasma at baseline, 1/4, 1, 3, 6, and 12 hours, and 3 and 12 hours after the infusion ended, and in daily 24-hour urine collections.

Reversal of megaduodenum and duodenal dysmotility associated with improvement in nutritional status in primary anorexia nervosa.

Anorexia nervosa is considered one type of eating disorder that may result in severe malnutrition. Patients with this disorder commonly complain of postprandial nausea, abdominal pain, and distension. We describe the radiologic and motility abnormalities associated with anorexia nervosa in a 21-year-old female.

Serious renal impairment is associated with long-term parenteral nutrition.

Thirty-three current long-term total parenteral nutrition (TPN) patients (13 men, 20 women) aged 21 to 79 years were prospectively studied to evaluate their change in glomerular filtration rate since beginning TPN. Creatinine clearance (CrCl) from the subject's initial home TPN clinic visit and at present were estimated from standard formulas and compared. The CrCl in 12 patients who had received home TPN for > 10 years was estimated retrospectively on a yearly basis.

Central venous catheter infection caused by Moraxella osloensis in a patient receiving home parenteral nutrition.

We report the first case of a central venous catheter infection caused by Moraxella osloensis, which was successfully treated without catheter removal. The isolation, identification, and pathogenesis of this species are discussed. It is recommended that Moraxella isolates be identified to species in order to determine the relative pathogenic and opportunistic roles of the various Moraxella species.

Quantitative electromyographic analysis of changes in muscle activity following botulinum toxin therapy for cervical dystonia.

Reports of efficacy of botulinum toxin for cervical dystonia have relied on subjective reports of improvement or various clinical rating scales. We studied 19 patients with cervical dystonia using Turns analysis to determine if quantitative EMG measures of muscle activity changed following botulinum toxin injections. Before and after botulinum toxin injections, six muscles were evaluated bilaterally.

Total serum amylase but not lipase correlates with measured glomerular filtration rate.

Elevated serum amylase and lipase levels have been reported in patients with renal failure. Because the diagnosis of pancreatitis is often based on elevations in serum amylase or lipase along with abdominal pain, we determined the relationship between total serum amylase, lipase, and glomerular filtration rate (GFR). Thirty-three subjects, 13 men and 20 women (age range, 21-79), were included in the study.

Multiple functions of nucleosomes and regulatory factors in transcription.

The in vivo packaging of DNA with histone proteins to form chromatin makes its transcription a difficult process. Biochemical and genetic studies are beginning to reveal mechanistic details of how transcriptional regulatory factors confront at least two hurdles created by nucleosomes, the primary structural unit of chromatin. Regulatory factors must gain access to their respective binding sites and activate the formation of transcription complexes at core promoter elements.

Low plasma free choline is prevalent in patients receiving long term parenteral nutrition and is associated with hepatic aminotransferase abnormalities.

Hepatic transaminase abnormalities have been previously reported in patients receiving long term total parenteral nutrition (PN). We sought to determine if such abnormalities are caused by choline deficiency-induced hepatocyte damage. In 41 subjects (19 male, 22 female) aged 45.

Late onset primary systemic carnitine deficiency exacerbated by carnitine-free parenteral nutrition.

We describe a 21-year-old male with previously normal plasma total and free carnitine levels who developed a deficiency manifest by decreased plasma and muscle total and free carnitine, decreased urine carnitine, severe hepatic steatosis, mediastinal lipomatosis, progressively impaired triglyceride clearance, myopathy and intermittent hypoglycemia. This case demonstrates that systemic carnitine deficiency may occur in some patients receiving long term carnitine-free TPN. Carnitine may be an essential element of the diet in this patient population.

Central venous catheter sepsis caused by unusual Gordona (Rhodococcus species: identification with a digoxigenin-labeled rDNA probe.

We describe central line sepsis caused by Gordona (Rhodococcus) species in two patients, which complicated receipt of long-term total parenteral nutrition at home. Species identification was attempted by conventional biochemical analysis and analysis of polymorphisms in the ribosomal RNA genes with use of a digoxigenin-labeled rDNA probe. Using these techniques, we identified our first patient's isolate as Gordona terrae.

Intrafamilial heterogeneity in hereditary motor neuron disease.

Although there are varied inheritance patterns in motor neuron disease (MND), the phenotype of MND is reported to be constant within these families, ie, cases of amyotrophic lateral sclerosis or primary lateral sclerosis do not occur in pedigrees with cases of spinal muscular atrophy. We describe four pedigrees whose members diverged in the phenotype of MND expressed. The intrafamilial variation of phenotype suggests a similar pathogenesis for some of the varied types of familial MND and the need for careful inquiry of family history in all patients with MND.

Iodine supplementation in children receiving long-term parenteral nutrition.

In 18 children receiving long-term total parenteral nutrition (TPN) without iodide supplements, thyroid function test results were normal but serum iodide levels were greater than in control subjects (p less than 0.01). Iodine contamination of TPN solutions and fat emulsions accounted for only half of the recommended parenteral intake.

Carnitine status of children receiving long-term total parenteral nutrition: a longitudinal prospective study.

Nine children receiving carnitine-free total parenteral nutrition for 7.2 +/- 2.6 years since birth were prospectively studied for 3 years.

Determining neuromuscular jitter using a monopolar electrode.

Neuromuscular jitter was determined in the extensor digitorum communis (EDC) of 41 healthy control subjects and 8 patients with myasthenia gravis (MG). Standard single-fiber electromyographic (SFEMG) techniques were used, except that a monopolar electrode (MPE) was substituted for a single-fiber electrode (SFE). In normals, mean jitter for 20 pairs was 22.

Botulinum toxin injection for spasmodic torticollis: increased magnitude of benefit with electromyographic assistance.

To determine the usefulness of EMG-assisted botulinum toxin (BOTOX) injections for the treatment of spasmodic torticollis (ST), we randomized 52 ST patients into two groups and studied them prospectively. In one group [(E+C)RX, N = 28], the muscles were selected for BOTOX injection using both clinical and EMG examination and then injected with EMG assistance. In the second group [(C)RX, N = 24] the muscles were selected for BOTOX injection based solely on clinical examination and injected without EMG assistance.

Lecithin increases plasma free choline and decreases hepatic steatosis in long-term total parenteral nutrition patients.

Plasma-free choline levels have previously been found below normal in patients receiving long term parenteral nutrition (TPN). In a group of 15 patients receiving home TPN who had low plasma free choline levels (6.3 +/- 0.