Parent and child mealtime behavior in families of children with cystic fibrosis.

OBJECTIVES: We investigated the hypothesis that children with cystic fibrosis (CF) and their parents would show more maladaptive behaviors during dinner than children without CF and their parents. STUDY DESIGN: Children with CF (n = 32) and their parents were compared with 29 children without CF and their parents on the rate and frequency of parent-child behaviors during a typical dinner in the families' homes by using multivariate analyses of variance. RESULTS: When the rate of behavior, controlling for meal length, was examined, no differences were found between groups. However, parents of children with CF were found to differ from parents of control subjects in the frequency of direct and indirect commands (P <.05), coaxes (P <.01), physical prompts (P <.01), and feeding their child (P <.05). Children with CF were found to engage in more talk, spend more time away from the table, refuse food, and exhibit more noncompliance toward commands to eat than control children (P <.05 for all child variables). When behaviors were examined as a function of meal phase, parents of children with and without CF both showed an increase in commands (P <.01), coaxes (P <.05), feeds (P <.01), and physical prompts (P <.01) in the second half of the meal as compared with the first. Children with CF and the control children showed an increase in behaviors incompatible with eating during the second half of the meal compared with the first (P <.01). When faster eaters were compared with slower eaters, faster eaters consumed a higher percentage of the recommended daily allowance of energy (P <.01) than slower eaters and showed a trend to be at higher weight percentiles for age and sex (P =.08) regardless of group (CF or control). CONCLUSIONS: Children with CF and their parents do not differ from children without CF and their parents in the rate of behaviors exhibited or types of strategies used to encourage eating. However, children with CF and their parents engage in these behaviors more frequently. Our data do not support typical parenting behaviors as effective in meeting the CF dietary requirements. Additional support in the form of child behavior management training may be needed to assist parents in meeting their child's caloric requirements.

Subcutaneous forearm transplantation of autologous parathyroid tissue in patients with renal hyperparathyroidism.

BACKGROUND: Parathyroidectomy is required in up to 5% of patients with chronic renal failure. Intramuscular transplantation of autologous parathyroid tissue in the forearm has been the traditional method of transplantation at the time of total parathyroidectomy. The removal of an intramuscular transplantation can be technically difficult should graft-dependent hyperparathyroidism (GRH) occur. This problem resulted in our initiating a study of subcutaneous transplantation with total parathyroidectomy in patients with renal failure. METHODS: Twenty-six patients who were receiving dialysis therapy underwent total parathyroidectomy and subcutaneous transplantation. Parathyroid tissue was diced into 1- to 2-mm pieces, and 6 pieces were grafted into 6 subcutaneous pockets of the forearm. Intact parathyroid hormone was measured within 48 hours of operation and in the bilateral antecubital veins 1 to 24 months after the operation to assess completeness of resection and graft function, respectively. RESULTS: No major surgical complications occurred. Symptoms improved in 24 patients (85%). Graft failure rate was 4.3%. No GRH was observed. Follow-up was 4 to 55 months (mean, 27 months). CONCLUSIONS: This study indicates that the subcutaneous transplantation function is comparable to intramuscular transplantation and suggests a decreased incidence of GRH. Subcutaneous transplantation is technically easier than intramuscular transplantation and has the additional advantage of easy removal should GRH occur.

Descriptive analysis of eating behavior in school-age children with cystic fibrosis and healthy control children.

STUDY OBJECTIVE: To investigate calorie intake, behavioral eating styles, and parent perception of eating behavior of school-age children with cystic fibrosis (CF) compared with healthy peers. DESIGN: A two-group comparison study. SETTING: A clinical sample of 28 school-age children with CF and a community sample of 28 healthy peers matched for age (6 to 12 years) and socioeconomic status. MEASUREMENTS AND MAIN RESULTS: The children with CF consumed more calories per day (2175 cal/d) than the control children (1875 cal/d) and achieved a significantly higher recommended daily allowance (RDA) of energy (128% of the RDA) than the control children (91.61% of the RDA). Fifty-four percent of the CF sample were achieving the CF dietary recommendations of 120% of the RDA. Despite this energy intake, the CF sample was significantly below the control sample on weight (24.56 vs 31.23 kg), height (125.48 vs 133.06 cm), and z score for weight (-0.811 vs 0.528) and height (-0.797 vs 0.371). On measures of behavioral eating style, the CF sample had significantly longer meals (23.90 min) than the control sample (17.34 min) and had a significantly slower pace of eating (43.27% 10-second intervals with bites) than the control sample (51.29% 10-second intervals with bites) but did not differ significantly on the number of calories consumed during dinner. On a measure of parent report of mealtime behaviors, parents of the children with CF rated mealtime behavior problems of "dawdles" and "refuses food" as more intense (mean, 3. 46) than did the parents of control children (mean, 2.67). For the CF sample, a significant correlation was found between the parent intensity ratings of problem behavior in general and meal duration (r = .48), and a significant negative correlation was found between the parent intensity ratings of problem mealtime behaviors and the percentage of intervals with bites (pace of meal) (r = -.533). CONCLUSIONS: Although the school-age children with CF were consuming more calories per day than their healthy peers, and more than 50% of the children in the CF sample were at or above the CF dietary recommendations, the children in the CF sample were significantly below the control children on measures of weight and height. The behavioral data suggest that increased caloric intake is not without cost, because the CF sample spent an additional 7 minutes per day at dinner and ate their meals at a slower pace than their healthy peers. These data were associated with higher intensity ratings of mealtime behaviors by parents of children with CF. These findings point to the need for individualized assessment of energy needs for school-age children with CF and comprehensive programs that teach parents behavioral strategies to motivate their children to meet these higher energy requirements in an adaptive manner.

Behavioral intervention to improve calorie intake of children with cystic fibrosis: treatment versus wait list control.

Changes in calorie intake and weight gain were evaluated in five children with cystic fibrosis (CF) who received behavioral intervention and four children with CF who served as wait list controls. The behavioral intervention was a 6-week group treatment that provided nutritional education plus management strategies aimed at mealtime behaviors that parents find most problematic. The control group was identified prospectively and was evaluated on all dependent measures at the same points in time pre- and posttreatment as the intervention group. Difference scores on calorie intake and weight gain from pre- to posttreatment were compared between groups using t tests for independent samples. The behavioral intervention group increased their calorie intake by 1,032 calories per day, while the control group's intake increased only 244 calories per day from pre- to posttreatment [t(6) = 2.826, p = 0.03]. The intervention group also gained significantly more weight (1.7 kg) than the control group (0 kg) over the 6 weeks of treatment [t(7) = 2.588, p = 0.03] and demonstrated catchup growth for weight, as indicated by improved weight Z scores (-1.18 to -0.738). The control group showed a decline in weight Z scores over this same time period (-1.715 to -1.76). One month posttreatment, the intervention was replicated with two of the four children from the control group. Improved calorie intake and weight gain pre- to posttreatment were again found in these children. At 3- and 6-month follow-up study of children receiving intervention, maintenance of calorie intake and weight gain was confirmed. No changes were found on pulmonary functioning, resting energy expenditure, or activity level pre- to posttreatment. This form of early intervention appears to be promising in improving nutritional status and needs to be investigated over a longer period of time to evaluate the effects of treatment gains on the disease process.

Pneumothorax in polysubstance-abusing marijuana and tobacco smokers: three cases.

Three patients are reported who suffered spontaneous pneumothorax, each of whom also had a history of daily marijuana and tobacco use. The patients ranged in age from 24 to 37 years, had smoked marijuana on a daily basis for 10 to 14 years, and had 11 to 50 pack-year tobacco-smoking histories. Marijuana may predispose to pneumothorax both by accelerating tobacco-induced lung disease, and by the frequent performance of the Valsalva maneuver during marijuana smoking. Although there is an association between pneumothorax and heavy tobacco use, an association with chronic daily marijuana use has not previously been reported.

Increasing calorie consumption in children with cystic fibrosis: replication with 2-year follow-up.

Three mildly malnourished children with cystic fibrosis and their parents participated in a behavioral group-treatment program that focused on promoting and maintaining increased calorie consumption. Treatment included nutritional education, gradually increasing calorie goals, contingency management, and relaxation training, and was evaluated in a multiple baseline design across four meals. Children's calorie intake increased across meals, and total calorie intake was 32% to 60% above baseline at posttreatment. Increased calorie consumption was maintained at the 96-week follow-up (2 years posttreatment). The children's growth rates in weight and height were greater during the 2 years following treatment than the year prior to treatment. Increases in pace of eating and calories consumed per minute were also observed 1 year posttreatment. These findings replicated and extended earlier research supporting the efficacy of behavioral intervention in the treatment of malnutrition in children with cystic fibrosis.

A behavioral approach to increasing calorie consumption in children with cystic fibrosis.

To increase calorie consumption of 5 mildly malnourished children with cystic fibrosis (CF), intervention was implemented in multiple baseline fashion across snack and three meals. Intervention involved nutritional education establishing gradually increasing calories goals, teaching parents contingency management strategies, and a reward system for achieving calorie goals. Following 6 treatment sessions, the children's calorie intake increased across meals and total calorie intake was 25 to 43% above baseline. The calorie increase was maintained at 9-month follow-up. Significant changes in weight and height were made during treatment and the year following intervention. The results suggest that the long-term oral intake of children with CF can be modified by a short-term behavioral intervention.

Cimetidine-induced interstitial nephritis with response to prednisone therapy.

Acute renal failure developed in a patient after she received cimetidine therapy. A renal biopsy specimen showed acute interstitial nephritis. Withdrawal of cimetidine therapy led to improvement of renal function. Rechallenge with cimetidine occurred during prednisone therapy. Renal function continued to improve, but worsened when the treatment with these steroids was discontinued.

Patient-reported compliance with cystic fibrosis therapy.

Parents and patients in the Rhode Island Cystic Fibrosis Center were interviewed to determine the extent of compliance with various aspects of the therapeutic regimen. Current patterns of compliance were compared with retrospective clinic records of reported compliance up to 8 years prior to the time of study. Actual clinic attendance and reported compliance with medications were high. Patients reported significantly lower compliance with diet and chest physiotherapy. Most patients reported less compliance with chest physiotherapy at the time of study than 3 to 8 years earlier, regardless of the severity of their lung disease. Patients frequently reported self-medication with non-prescribed vitamins, antibiotics, or other drugs. Patients' perceptions of the severity of their disease and the possible adverse consequences of discontinuing therapy probably contribute to both good medication compliance and self-medication.

Tuberculous mediastinal lymphadenitis presenting as chest wall mass.

Tuberculous mediastinal lymphadenitis is an unusual presentation of primary tuberculosis in adults. In the case reported here, the mediastinal focus was initially obscured by a large chest wall mass that communicated with it. This combination of findings, which we believe to be reported first here, underscores the need to consider tuberculosis in differential diagnosis of mediastinal and chest wall masses.

Respiratory distress and hypoxemia in systemic mastocytosis.

A 25-year-old woman with documented mastocytosis developed hypoxemia with pruritus, diarrhea, headache, and hypotension on two separate occasions. The hypoxemia appeared to be related to a massive release of histamine. Resolution of the patient's symptoms was accompanied by the return of her arterial oxygen tension to normal levels.

Isolation and characterization of two glycoproteins from patients with alveolar proteinosis.

The saline-insoluble particulate material obtained by pulmonary lavage from four patients with alveolar proteinosis was analyzed for total lipid (52%), protein (44%), and carbohydrate (4%). Sodium dodecyl sulfate-gel electrophoresis revealed only three major peptides in these patients. Molecular weights (from the gels) were 69,000, 62,000, and 36,000, and the latter two peptides were periodic acid-Schiff stain-positive. Amino-acid and sugar analyses were performed on the peptides cut from the gels and on peptides purified by Sephadex G-200 chromatography. The 69,000-molecular-weight peptide contained no carbohydrate. Antibody studies and aminoacid analysis indicated that it was albumin. The 62,000-molecular-weight peptide contained 1% hydroxyproline, 1% hydroxylysine, 10% glycine, and 9% carbohydrate. The 36,000-molecular-weight peptide contained 1.2% hydroxyproline, 1% hydroxylysine, 13% glycine, 1.4% sialic acid, 2.6% glucose, 2.4% galactose, 2% mannose, 0.8% fucose, 0.4% glycosamine, and 0.6% galactosamine. Proteins extracted from kidney glomeruli with similar amino-acid and carbohydrate composition have been observed previously.