Acute cardiac disease in a patient with hyper-IgE syndrome.

SUMMARY: We describe the case of a 24-year-old male with hyper-IgE syndrome (HIES) which was diagnosed at 4 years of age and died from a very rare cardiac complication. He had typical clinical and laboratory manifestations of HIES, including total serum IgE as high as > 100,000 IU/mL. Stem cell transplantation was not available. During the 20-year follow-up, he suffered numerous various infections of the skin and deep organs, partial lung resection, as well as multiple bone fractures. At age 24, he developed acute decompensated heart failure associated with elevated serum troponin I and brain natriuretic protein. Two-dimensional echocardiogram revealed global hypokinesis of the left ventricle with estimated ejection fraction 20-25%, and catheterization revealed ectasia of multiple coronary arteries. Endomyocardial biopsy showed lymphocytic myocarditis, focal necrosis, mild fibrosis, and myxoid degeneration, but cultures were negative. The patient improved on corticosteroid therapy and was discharged on heart failure therapy and external defibrillator. Six weeks later, he developed supraventricular tachycardia and persistent global hypokinesis and was treated with amiodarone. A trial of intravenous immunoglobulin was initiated and was repeated as outpatient every four weeks for four times. However, his cardiac function did not improve and he developed severe hypotension and pulseless electrical activity arrest. Resuscitation was unsuccessful. To the best of our knowledge, this is the first reported case of HIES complicated with lymphocytic myocarditis. Both immunologists and cardiologists need to be aware of such a complication and practice caution in using immunosuppressants when the patient's immune status is markedly compromised.

Reading ability of parents compared with reading level of pediatric patient education materials.

OBJECTIVES: To test the reading ability of parents of pediatric outpatients and to compare their reading ability with the ability necessary to read commonly used educational materials; to compare individual reading grade levels with the levels of the last grade completed in school; and to further validate a new literacy screening test designed specifically for medical settings. DESIGN: Prospective survey. SETTING: Pediatrics outpatient clinic in a large, public university, teaching hospital. PARTICIPANTS: Three hundred ninety-six parents or other caretakers accompanying pediatric outpatients. MEASUREMENTS: Demographics and educational status were assessed using a structured interview. Reading ability was tested using the Rapid Estimate of Adult Literacy in Medicine (REALM) and the Wide Range Achievement Test-Revised. Written educational materials were assessed for readability levels with a computer program (Grammatik IV). RESULTS: The mean score on the REALM for all parents placed them in the seventh to eighth grade reading range, despite the mean self-reported last grade completed in school being 11th grade 5th month. Wide Range Achievement Test-Revised scores correlated well with REALM scores (0.82). Eighty percent of 129 written materials from the American Academy of Pediatrics, the Centers for Disease Control, the March of Dimes, pharmaceutical companies, and commercially available baby books required at least a 10th grade reading level. Only 25% of 60 American Academy of Pediatrics items and 19% of all materials tested were written at less than a ninth grade level, and only 2% of all materials were written at less than a seventh grade level. CONCLUSION: This study demonstrates that parents' self-reported education level will not accurately indicate their reading ability. Testing is needed to screen at-risk parents for low reading levels. In a public health setting, a significant amount of available parent education materials and instructions require a higher reading level than most parents have achieved. In such settings, all materials probably should be written at less than a high school level if most parents are to be expected to read them. The REALM can easily be used in busy public health clinics to screen parents for reading ability.

Parent comprehension of polio vaccine information pamphlets.

BACKGROUND: Medical information pamphlets often are written using language that requires a reading level higher than parents of many pediatric patients have achieved. Anecdotal reports suggest that many parents may not readily understand the federally mandated Public Health Service vaccine information pamphlets prepared by the Centers for Disease Control and Prevention (CDC) in 1991. The level at which the pamphlets need to be written for low-reading-level parents is undetermined, as is whether parents reading at higher levels will accept low-reading-level materials. METHODS: To determine whether a simple pamphlet prepared at a low reading level using qualitative and adult education techniques would be preferable to the available CDC polio vaccine information pamphlet, we conducted an integrated qualitative-quantitative study. We compared the parent reading time and comprehension of a simplified pamphlet (Louisiana State University, LSU) comprising 4 pages, 322 words, 7 instructional graphics, and a text requiring a 6th grade reading ability with the equivalent 1991 CDC vaccine information pamphlet comprising 16 pages, 18,177 words, no graphics, and a text requiring a 10th grade reading level. We measured the reading ability of 522 parents of pediatric patients from northwest Louisiana seen at public clinics (81%) and in a private office (19%). Of the entire group, 39% were white, 60% African-American, and 1% Hispanic; the mean age was 29 years; the mean highest grade completed was 12th grade 3 months; and the reading level was less than 9th grade in 47% of parents and less than 7th grade in 20%. After parents were given one of the pamphlets to read, their reading time, comprehension, and attitude toward the pamphlet were measured. RESULTS: Mean comprehension was 15% lower for CDC than for LSU (56% vs 72% correct; P < .001) and reading time was three times longer for CDC than for LSU (13 minutes 47 seconds vs 4 minutes 20 seconds; P < .0001). These trends were significant for parents reading at all but the lowest levels. Mean comprehension and reading time did not differ among parents reading at the third grade level or less. However, mean comprehension was greater and reading time lower for LSU among parents at all reading abilities greater than the third grade. Parents in the private practice setting took the longest time to read the CDC (20 minutes 59 seconds vs 5 minutes 46 seconds, LSU), yet their comprehension on the LSU was significantly higher than on the CDC (94% vs 71%; P < .0001). Two focus groups of high-income parents were unanimous in preferring the LSU. CONCLUSIONS: A short, simply written pamphlet with instructional graphics was preferred by high- and low-income parents seen in private and public clinics. The sixth grade reading level appears to be too high for many parents in public clinics; new materials aimed at third to fourth grade levels may be required. The new 1994 CDC immunization materials, written at the eighth grade level, may still be inappropriately high. The American medical community should adopt available techniques for the development of more effective patient-parent education materials.

Respiratory syncytial virus infection among intubated adults in a university medical intensive care unit.

Respiratory syncytial virus is the major cause of lower respiratory tract infection in children. Adults who are immunocompromised, aged, institutionalized, and/or have underlying medical diseases may be at risk for severe RSV infection. Intubated adults in an MICU were evaluated for evidence of RSV infection. Respiratory secretions were analyzed by cell culture and RSV EIA. Serologic testing was obtained. Respiratory secretions from MICU personnel with acute respiratory symptoms and patients admitted for pneumonia, asthma, or COPD also were screened. Five of 11 intubated patients had evidence of RSV infection. One of seven MICU employees and four of 48 ward patients had RSV-positive respiratory secretions. During community outbreaks of RSV infection, adults admitted to an MICU already may be infected with RSV; those admitted for other reasons are at risk for nosocomial infection. Patients occupying other hospital units and personnel may be instrumental in the nosocomial dissemination of RSV.

Failure of erythromycin ointment for postnatal ocular prophylaxis of chlamydial conjunctivitis.

Chlamydia trachomatis is the most common pathogen associated with conjunctivitis during early infancy in the United States. During a 13-month interval at our medical center 4834 infants were born, 311 of whom (6.4%) had conjunctival specimens tested for chlamydial antigen before the age of 12 weeks. In 44 (14% of all tested infants, 0.9% of live births) chlamydial antigen was present. Because the rate of asymptomatic maternal chlamydial endocervical colonization is estimated to be 26% at our institution (previous prospective study), we calculated a minimal failure rate for erythromycin ocular prophylaxis of from 7 to 19.5%. A subsequent case-control study revealed that mothers of infants with chlamydial conjunctivitis were more likely to be primiparous (P = 0.03) and experience longer duration of rupture of membranes before delivery (P = 0.046). We conclude that a substantial percentage of infants exposed to Chlamydia develop chlamydial conjunctivitis despite receiving erythromycin ocular prophylaxis.

Selective use of vancomycin to prevent coagulase-negative staphylococcal nosocomial bacteremia in high risk very low birth weight infants.

OBJECTIVE: To determine whether vancomycin added to parental nutrition (PN) fluids could prevent nosocomial infections in very low birth weight newborns and which infants would benefit most from prophylaxis. DESIGN: Double blind, randomized controlled study. SETTING AND STUDY POPULATION: Very low birth weight infants receiving PN in a tertiary neonatal intensive care unit. METHODS: Thirty-eight infants with and without central vascular catheters were randomized to receive no medication or 25 microg/ml vancomycin added to PN for the duration of the infant's PN requirements. RESULTS: The addition of 25 microg/ml vancomycin to PN prevented bacteremia in very low birth weight infants receiving PN. There was a significant reduction in the number of coagulase-negative staphylococcal (CONS) bacteremias (defined as isolation of the same organism from two positive blood cultures) during PN (5 vs. 0; P = 0.037) as well as the total number of bacteremias and fungemias (9 vs. 1; P = 0.036). The total number of hospital days (108 +/- 13 vs. 76 +/- 6; P = 0.039) were reduced in infants receiving vancomycin. Infants with birth weights of < 1000 g who received corticosteroids for treatment of chronic lung disease benefitted most from treatment. No vancomycin-resistant strains of CONS or enterococci were detected during the study period. CONCLUSIONS: Prophylactic treatment with vancomycin effectively prevented CONS bacteremia under the conditions of the study. Its use was most effective in infants with birth weights of <1000 g.

Comparison of two selective media developed to isolate Pseudomonas cepacia from patients with cystic fibrosis.

Two media selective for Pseudomonas cepacia have been described recently: OF-polymyxinbacitracin-lactose agar (OFPBL) and P. cepacia agar (PCA). We compared these by culturing sputum from 27 patients with cystic fibrosis. Sputum from each patient was studied using streak-plate (SP) and quantitative (Q) culture methods. Five strains of P. cepacia were isolated from four patients (15%). P. cepacia was not found on routine media by SP or Q methods in three of the four patients. All five isolates grew on both OFPBL and PCA and all were recovered by SP and Q culture methods. Colony counts of P. cepacia obtained by Q cultures were similar on both selective media. Each of the selective media allowed the growth of other organisms; nineteen of 27 specimens cultured on OFPBL yielded non-P. cepacia spp. and six of 27 specimens cultured on PCA yielded non-P. cepacia spp. species (p less than 0.005). Pseudomonas aeruginosa was isolated from 26 of 27 patients on routine media (96%). This organism was recovered from four specimens cultured on OFPBL but from none on PCA (p less than 0.05). OFPBL and PCA are both selective for P. cepacia and enhance the ability to recover this organism from patients with cystic fibrosis using either SP or Q cultures of sputum; however, PCA is significantly more inhibitory for non-P. cepacia spp. than OFPBL.

Single-dose pharmacokinetics of bupropion in adolescents: effects of smoking status and gender.

Sustained-release (SR) bupropion (Zyban) is approved as a smoking cessation aid for adults. Since smoking often begins in adolescence, we determined the single-dose pharmacokinetics of bupropion SR in 75 adolescent subjects ranging from 13 to 18 years old. Subjects self-reported their smoking status. Urinary cotinine concentration was used to verify smoking status. Thirty-seven subjects (18 males, 19 females) were classified as cigarette smokers and 38 were nonsmokers (19 males, 19 females). Fasted subjects received one tablet (150 mg) of bupropion SR, and plasma samples were collected before (0) and 1/2, 1, 2, 3, 4, 6, 8, 24, 48, and 72 hours after dosing. Plasma samples were analyzed for bupropion and its three major metabolites (hydroxybupropion and the aminoalcohol isomers, erythrohydrobupropion plus threohydrobupropion, expressed as a composite) by solid-phase extraction, followed by LC/MS/MS. Factorial analysis of variance (ANOVA) was used to evaluate the effects of smoking and gender on pharmacokinetic parameters. Smokers and nonsmokers differed significantly (p < 0.05) in age and urinary cotinine (p < 0.01) concentration but did not differ significantly in mean weight, height, body surface area, or body mass index. The pharmacokinetic (PK) parameters for bupropion and hydroxybupropion did not differ between smokers and nonsmokers, but differences were found between male and female subjects. Mean values for area under the plasma concentration versus time curve (AUC0-->infinity), volume of distribution (Vd beta) normalized to body weight, maximum plasma concentration (Cmax), and elimination half-life (t1/2 beta) for bupropion were significantly (p < 0.05) greater in females than males, while clearance of bupropion normalized to body weight (CL/f) did not differ between males and females. Females also exhibited significantly (p < 0.05) larger values for hydroxybupropion mean AUC0-->infinity and Cmax than males. The mean ratio of hydroxybupropion to bupropion AUC for adolescents was approximately 4 to 5, which is lower than that previously reported for adults. In conclusion, smoking status does not affect the single-dose pharmacokinetics of bupropion SR in adolescents. However, females differ from males in several potentially important PK parameters for bupropion and its major metabolite, hydroxybupropion.

Fournier's gangrene in children.

Necrotizing fasciitis of the genitalia is a rare urologic emergency that is especially uncommon in children. We report a case of Fournier's gangrene in a four-year-old boy and analyze the data from 55 previously reported cases. Pediatric cases have been successfully managed with a more conservative surgical approach and have had a significantly lower mortality rate than adult cases.

Low literacy and violence among adolescents in a summer sports program.

PURPOSE: To investigate the relationship between inadequate literacy and violent behavior among adolescents. METHODS: This descriptive study involved a convenience sample of 386 adolescents who participated in a summer track and field and literacy program serving youths in low-income neighborhoods in Shreveport, Louisiana, during 1994-1996. Self-reported violence was measured using the Youth Risk Behavior Survey (YRBS) and reading grade levels were measured by the Slosson Oral Reading Test-Revised (SORT-R). RESULTS: Youths ranged in age from 11 to 18 years; 66% were male, and 86% were African-American. Forty-three percent of adolescents tested had below-grade reading levels (> or = 2 grades). Participants with below-grade reading skills had higher rates of self-reported violent behaviors compared with those reading at grade level. When gender, race, and age were controlled for, adolescents reading below grade level were significantly more likely to report carrying weapons [odds ratio (OR) = 1.9; 95% confidence interval (CI) 1.1-3.5], carrying guns (OR = 2.6; CI 1.1- 6.2), to have been in a physical fight at school (OR = 1.7; CI 1.1-2.6), and to have been in a physical fight resulting in injuries requiring treatment (OR = 3.1; CI 1.6-6.1). In addition, youths reading below grade level were significantly more likely to be threatened at school with a weapon (OR = 2.1; CI 1.2-3.7) and to report missing days of school in the previous 30 days because they felt unsafe at school (OR = 2.3; CI 1.3-4.3). In characterizing the violence related behaviors, we found that low reading-level adolescents were more likely to be both aggressor/perpetrator and victim (44% vs. 32%; p = .02) and less likely to be only a victim (6% vs. 12%; p = .04) compared to adolescents with grade-appropriate reading skills. CONCLUSIONS: Below-grade-level reading was significantly related to violence behaviors among adolescents who volunteered for a summer track and field program. Longitudinal studies are needed to further investigate the relationship of below-grade-level reading and aggressive/perpetrator and victim behaviors.

Tobacco use among male high school athletes.

PURPOSE: The purpose of this study was to compare tobacco use among high school male athletes with their nonathlete counterparts. We hypothesized that there was an inverse correlation between the intensity level of the sport and frequency of tobacco use. METHODS: Students were surveyed at seven high schools in northwest Louisiana using a 109-item questionnaire. Of the 1,200 males tested, 83% participated in one or more sports. The mean age was 15.8, and mean grade level was 10th. Sixty-seven percent were white, 27% African-American (AA), and 6% other. RESULTS: Forty-one percent of the adolescent males tested were one or more tobacco products, 31% reported cigarette smoking, 21% chewed tobacco, and 18% used snuff. Eleven percent reported using all three tobacco products. Race was a significant determinant of tobacco use, with whites being more likely to use each of the three tobacco products (P < .001). Medium- and high-intensity athletes were significantly (P < .01) less likely to be heavy smokers than athletes participating in low-intensity sports and nonathletes. However, athletes of each intensity sport used chewing tobacco and snuff at significantly higher rates (P < .001) than nonathletes. When race and grade point average were controlled, sports intensity was a significant predictor of smokeless tobacco use but not overall smoking behavior. Both AA and white high school male athletes at all sport intensity levels were using chewing tobacco and snuff at a rate higher at least 1.5 times that of their nonathlete counterparts. CONCLUSIONS: In our study, high school males' sports participation was a predictor of smokeless tobacco use but not overall smoking behavior. Although the probability of AA high school athletes using smokeless tobacco was low compared to whites, the pattern of use was similar across intensity levels of sports.

A polio immunization pamphlet with increased appeal and simplified language does not improve comprehension to an acceptable level.

We used a randomized trial to compare two polio vaccine pamphlets written on a sixth grade level--the vaccine information statement prepared by the Centers for Disease Control (CDC) and an easy-to-read pamphlet we developed (LSU)--for reading ability, comprehension and preference among 610 parents with a broad range of demographic characteristics. Parents at all reading levels and incomes preferred LSU (76% vs. 21%, P < 0.001). Although readers of LSU achieved significantly higher comprehension (65% vs. 60%, P < 0.05) this difference may not be clinically significant. The information items presented with instructional graphics were the only items on which differences in comprehension levels achieved both clinical and statistical significance. Comprehension was lowest for the CDC mandated information on risks and the National Injury Compensation. Our findings demonstrate that simplifying written immunization material and making it more suitable will increase appeal, but such modification may not raise comprehension to an acceptable level without use of instructional graphics. Health education materials intended for general parent populations, which are written on a sixth grade reading level, may not adequately educate parents or prepare them for a discussion with their physicians.

Treatment of Chlamydia trachomatis identified with Chlamydiazyme during pregnancy. Impact on perinatal complications and infants.

A rapid enzyme immunoassay antigen detection system (Chlamydiazyme) was used to screen 199 asymptomatic, third-trimester women. Fifty-two (26%) were Chlamydiazyme positive; they were mostly primiparous, single, young and black. Infected women were offered erythromycin therapy, counseling and posttherapy retesting. Sexual partners were treated likewise. Erythromycin compliance, determined by a questionnaire, was high (92%), and side effects (16%) were tolerable. Pregnancy outcome and infant illnesses were monitored to determine the effectiveness of therapy. There were no significant differences in pregnancy outcome in the Chlamydiazyme-negative and treated, Chlamydiazyme-positive women. Prospective evaluation of infants born to 48 negative and 50 treated, Chlamydiazyme-positive women revealed no significant differences in the incidence of respiratory tract illnesses or conjunctivitis. Chlamydiazyme can be used in a screening program to identify and treat third-trimester women infected with C trachomatis. As previously reported, erythromycin therapy for colonized women interrupted the expected transmission of C trachomatis to their infants.

The role of the capsular polysaccharide in the activation of the alternative pathway by the pneumococcus.

Previous studies have shown that when pneumococci are incubated in normal, nonimmune serum, they activate the alternative pathway and opsonically active C3b is fixed to the surface of the organism. Other studies have demonstrated that C3-dependent opsonization via the alternative pathway plays a significant role in the nonimmune host's defense against the pneumococcus. The present studies concern the role of the capsular polysaccharide in initiating the activation of the alternative pathway by the pneumococcus. Some pneumococcal capsular polysaccharide types, but not all, are able to activate the alternative pathway. Soluble purified capsular polysaccharide types 1, 4 and 25 activate the alternative pathway, whereas types 2, 3, 14, and 19 do not. Since the capsular polysaccharides exist in their native form attached to the pneumococcal surface, selected capsular polysaccharides were also tested for their ability to activate the alternative pathway when attached to a particulate carrier, sheep erythrocytes. Capsular polysaccharide types 2 and 3 failed to activate the alternative pathway when attached to sheep erythrocytes, paralleling the results obtained when these capsular polysaccharides were in solution. In contrast, the type 25 capsular polysaccharide not only activated the alternative pathway when attached to sheep erythrocytes, as it had when in solution, but it also initiated alternative pathway-mediated lysis of the erythrocytes. The capsular polysaccharide is not required for the activation of the alternative pathway by the pneumococcus. Although all types of encapsulated pneumococci are able to activate the alternative pathway, not all the purified capsular polysaccharide types are able to do so. In addition, a nonencapsulated pneumococcus, derived originally from a type 2 organism, activates the alternative pathway as well as a fully encapsulated type 2 pneumococcus.

Summertime respiratory syncytial virus infection: epidemiology and clinical manifestations.

Respiratory syncytial virus (RSV) is a common serious pathogen known to produce annual winter epidemics in young children. A 2-year study of children with significant respiratory disease during the summer revealed a 21% incidence of RSV infection. Respiratory secretions collected from ill children in the LSUMC outpatient clinics, from children seen by private physicians, and from children hospitalized with respiratory tract disease were assayed for RSV antigens. Approximately 39% of those surveyed in 1987 and 13% of those studied in 1988 were positive. As this prevalence was significant, we compared RSV-induced disease in 20 patients hospitalized in summer and 20 hospitalized in winter (1989). The patients were matched by age, weight, sex, and race. Comparisons included subjective severity of disease, presenting symptoms, physical findings, chest roentgenograms, treatment, and average length of hospital stay. No significant differences in disease severity and/or clinical presentation were found. Our findings show that RSV induces disease in the summertime more frequently than generally recognized, and severe disease requiring hospitalization is not infrequent. Physicians should consider RSV in children with serious respiratory disease throughout the year.

Mucocutaneous lymph node syndrome in North Louisiana: review of 15 cases.

Mucocutaneous lymph node syndrome (MLNS) is being more frequently reported in the United States, and it appears to be an important disease of childhood. Fifteen infants and children with MLNS were treated at the Louisiana State University teaching hospitals in Shreveport and Monroe between August 1978 and January 1981. A cluster of nine of the cases (60%) occurred between February and May 1980. In this series the male to female ratio was 2:1, and 53% of the patients were between 11 and 15 months of age. In five of the patients, platelet counts and erythrocyte sedimentation rates were monitored for at least four weeks.

Effect of elevated glucose concentrations on cellular lipid peroxidation and growth of cultured human kidney proximal tubule cells.

This study has examined whether elevated glucose can induce lipid peroxidation and contribute to the inhibition of cell growth in human kidney proximal tubule(HPT) cells. HPT cells were cultured in media containing glucose concentrations of 8 mM (control), 25 mM, and 50 mM. Lipid peroxidation was assessed by the thiobarbituric acid reactivity and cell growth was assessed by 3H-thymidine uptake. Results show decreased (59%, p < 0.01) growth of HPT cells cultured in 50 mM glucose. Cells cultured in 50 mM mannitol did not show any growth inhibition, suggesting that the decreased cell growth associated with glucose is not due to osmolarity changes. There was an increase (108%, p < 0.02) in lipid peroxidation in cells cultured with high levels of glucose (50 mM) compared with controls and cells cultured with 50 mM mannitol. To examine if membrane lipid peroxidation or malondialdehyde (MDA, an end product of lipid peroxidation) has any role in the inhibition of cell growth, we examined the effect of tertiary butylhydroperoxide (TBH, known to cause lipid peroxidation and generate MDA) on the growth of HPT cells. TBH decreased cell growth (49, 17 and 3% of controls at 0.1, 0.25, and 0.5 mumole TBH/ml medium). Similarly, a marked reduction in the growth was observed with exogenous MDA (72, 69 and 34% of controls at 0.1, 0.25, and 0.5 mumole MDA/ml medium). This suggests that elevated glucose can induce membrane lipid peroxidation and accumulation of MDA, which in turn can inhibit cellular growth and contribute to the altered structure and function of HPT cells in diabetes.