Family functioning in school-age children with cystic fibrosis: an observational assessment of family interactions in the mealtime environment

OBJECTIVE: To examine, using direct observation methodology, differences in family functioning at mealtime between families of school-age children with cystic fibrosis (CF) and families of school-age children without a chronic illness. METHOD: Family functioning was rated using the McMaster Mealtime Interaction Coding System (MICS) during a videotaped dinner among 28 families of children with CF and 27 families of non-ill, age-matched peers. Families were rated on overall family functioning and on six dimensions of the MICS: task accomplishment, communication, affect management, interpersonal involvement, behavior control, and role allocation. RESULTS: Ratings for families of a child with CF were significantly lower than they were for comparison families on overall family functioning and on four of the six MICS dimensions: communication, affect management, interpersonal involvement, and behavioral control. Moreover, a significantly greater percentage of families of children with CF were rated in the unhealthy range on overall family functioning and on five of six MICS dimensions. There was no relationship between family functioning and child weight status for children with CF. CONCLUSIONS: The current study suggests that for families of school-age children with CF, the family system is negatively affected during mealtime. Dietary interventions need to address family-centered, as well as child-centered, interventions to help families manage challenges presented during the family meal.     

Regulation of intracellular pH in cultured bovine retinal pigment epithelial cells

Regulation of intracellular pH (pH_i) in bovine retinal pigment epithelium (RPE) was investigated in cell culture. pH_i was measured using the pH-sensitive absorbance of intracellularly trapped 5 (and 6)-carboxy-dimethyl-fluorescein (CDMF). (1) Regulation of pH_i after induction of an acid load by removal of NH₄Cl could be blocked either totally by removal of extracellular sodium, or subtotally (about 90%) by application of amiloride (1 mmol/l). Additional flux measurements revealed a dose-dependent, amiloride-sensitive²²Na⁺-uptake into Na⁺-loaded cells. Both results suggest the presence of a Na⁺/H⁺ antiport.(2) When alkalinization of the cells was induced by preincubation with 50 mmol/l acetate in HCO ₃ ^– -Ringer's and subsequent removal of the weak acid, the following regulation was dependent on the presence of extracellular chloride. This process could be blocked with DIDS (1 mmol/l), suggesting the presence of a Cl^–/HCO ₃ ^– exchange mechanism.(3) We found no evidence for a Na⁺/HCO ₃ ^– -cotransport, which had been postulated to be present in RPE by others. We conclude that two processes are involved in regulation of pH_i in RPE: A Na⁺/H⁺ antiport responsible for recovery of pH_i from acid load, and a DIDS-sensitive Cl^–/HCO ₃ ^– exchange mechanism responsible for recovery of pH_i after alkalinization.Parts of this work jhave been published in abstract from [20, 21]     

Model system for isolation of competent ovarian-carcinoma cells from fresh tumor-tissue by a magnetic separation technique (macs)

Flow cytometric analysis of tumor cells in carcinomas is hampered by the presence of a variety of different cells in the tumor tissue and the surrounding stroma. To obtain single competent tumor cells, we have established a model system which can be applied to separate living cells from fresh ovarian carcinoma tissue. Due to the lack of tumor-cell surface specific antibodies, we isolated tumor cells by a procedure called 'negative tumor cell selection'. For this purpose, fresh ovarian carcinoma tissue, immediately after surgery, was subjected to mechanical disintegration using an automated mincing device to obtain a single-cell suspension (approximately 10(7) cells/g). Collagenase D (0.005%) was added to prevent further aggregation. Cells other than tumor cells were then labeled with a set of monoclonal antibodies directed to cell surface antigens: CD3 (T-cells), CD14 (monocytes), CD15 (granulocytes), CD45R (T-/B-cells) and 5B5 (fibroblasts). Anti-isotype antibodies coupled to ferrit microbeads were then reacted with the cell suspension and those cells reacting with the microbeads retained on a steel wool matrix in a magnetic field (1). Tumor cells not reacting with the microbeads were recovered by a simple wash of the steel wool matrix. All incubation steps were at 4 degrees C. This procedure, which takes about 2 hours, enables fast and simple isolation of single, living competent tumor cells from fresh tumor tissue and also from ascitic or pleuritic effusions. In a model system with cultured ovarian carcinoma cells and human leukocytes, tumor cell purity was about 93% and about 97% when re-subjected to the same procedure (respective recovery rates 75% and 50%). The still unlabeled tumor cells can subsequently be analyzed by flow cytometry or by central laser scanning microscopy for the presence of various surface antigens including receptors for proteases or growth factors. Moreover, after detergent treatment and fixation, flow cytometric multiparameter analysis such as simultaneous labeling of intracellular and surface antigens as well as nuclear DNA staining for ploidy and S-phase determination becomes possible.     

Generalized Smooth Muscle Hamartoma with Multiple Congenital Anomalies without the “Michelin Tire Baby” Phenotype

We report a patient with generalized smooth muscle hamartoma who presented with many of the variety of congenital anomalies that have been reported in babies with multiple symmetric circumferential rings of folded skin known as Michelin tire baby (MTB) syndrome, but our patient did not show the MTB phenotype. This constellation of findings in the absence of the MTB phenotype has not been previously reported.     

The Impact of Behavioral Intervention on Family Interactions at Mealtime in Pediatric Cystic Fibrosis

The current study evaluated the impact of a behavioral intervention (Be In Charge!), targeting caloric intake and weight gain in children with CF, on family interactions at mealtime. Forty-five families of children with cystic fibrosis (CF), ages 4 to 12 years, were randomized to Be In Charge! or nutrition education and assessed using the McMaster Mealtime Family Interaction Coding System. No differences were found in family functioning between the two interventions pre- to posttreatment or 1-year follow-up, except for Affect Management. A significantly greater percentage of families receiving Be In Charge! demonstrated improvement in Affect Management from pretreatment to 1-year follow-up. Implications for developing the next generation of behavioral interventions are discussed.     

The effects of weight management programs on self-esteem in pediatric overweight populations

OBJECTIVE: Review published findings on self-esteem and pediatric overweight, and changes in self-esteem subsequent to weight management programs. METHODS: We used PsycInfo and MedLine searches to identify peer-reviewed journal articles examining self-esteem changes following participation in weight management programs. RESULTS: Data regarding the relationship between self-esteem and obesity is mixed. Factors that place overweight children "at-risk" for low self-esteem include early adolescence, female gender, identification with majority cultural standards of body shape, exposure to teasing and peer victimization, a history of greater parental control over feeding, and internal attributions about weight status. Data from intervention studies suggest positive effects on self-esteem across settings. Components related to self-esteem improvements include weight change, parent involvement, and group intervention format. CONCLUSIONS: Well-designed, longitudinal studies using multidimensional measures of self-esteem, and following CONSORT guidelines are needed to confirm and expand these findings. Emphasis should be placed on examining mediators and moderators of self-esteem change.     

Signalling pathways identified in salivary glands from primary Sjögren’s syndrome patients reveal enhanced adipose tissue development

A characteristic feature of primary Sjögren’s syndrome (pSS) is the destruction of salivary and lacrimal glands mediated by mononuclear cell infiltration. Adipocytes can also occupy a large portion of the salivary gland (SG) tissue area, although little is known about their significance in pSS. We have previously investigated adipose tissue infiltration in SG biopsies from pSS patients and non-SS sicca controls. Our findings indicated the distinct incidence of adipose tissue replacement in pSS patients, where adipocytes were detected in interleukin (IL) 6 rich regions. We now aimed to examine the development of adipocytes in the SG microenvironment, and delineate their possible involvement in immune reactions. A microarray analysis was performed on SG from 6 pSS patients and 6 non-SS controls, where the expression levels of genes involved in adipose tissue development, inflammatory responses, and lymphoma development were assessed. Real-time PCR was carried out on SG from 14 pSS patients and 15 non-SS controls to account for IL6, IL10, and IL17 mRNA levels. Immunohistochemical staining of frozen SG tissue using IL17 was also conducted. Our results indicate signalling pathways identified in SG of pSS patients displayed genes leading to prominent adipose tissue development and reduced mitochondrial fatty acid beta-oxidation (ARID5B, OXCT1, BDH1, SOX8, HMGCS2, FTO, ECHS1, PCCA, ACADL and ACADVL), inflammatory responses (IL1R1, IL7R, IL10RA, IL15, IL18RAP, CCL2, CCL5, CCL22, CXCR6, CD14, and CD48), and lymphoma development via JAK-STAT signalling (STAT2, TYK2, EBI3, FAS, TNFRSF1B, MAP3K8, HMOX1, LTB, TNF, STAT1, and BAK1). Genes involved in interferon production and signalling were also detected (IRF1, IRF9, and IRF7), in addition to IL6, IL10, and IL17. Higher mRNA levels of IL6, IL17 and IL10 were observed in the SG of pSS patients compared to controls. Moreover, IL17 positive cells were detected mostly interstitially in the SG and around adipocytes, also within the focal infiltrates. In conclusion, adipocyte development seems to be more prominent in the SG of pSS patients, where adipose tissue replacement is also evident. Whether this is due to disease progression, or the repair process, remains to be investigated. Detection of IL17 positive adipocytes in the target organ suggests their involvement in immune reactions.     

Oral disorders,saliva secretion,and oral health‐related quality of life in patients with primary Sjögren's syndrome

Chemosensory function, burning sensations in the tongue (BST), halitosis, saliva secretion, and oral health‐related quality of life (OHRQoL) were investigated in patients with primary Sjögren's syndrome (pSS). In 31 patients with pSS and 33 controls, olfactory and gustatory functions were evaluated. Self‐reported complaints of dysgeusia, BST, and halitosis were recorded. Saliva secretion rates were measured and OHRQoL was assessed using the short‐form Oral Health Impact Profile (OHIP‐14). Patients had significantly lower olfactory (8.8 ± 3.5 vs. 10.7 ± 1.2) and gustatory (18.9 ± 7.1 vs. 25.4 ± 4.3) scores than controls, and significantly more patients complained of dysgeusia (58.1% vs. 0%), BST (54.8% vs. 6.1%), and halitosis (41.9% vs. 0%). A significantly greater proportion of patients with pSS had ageusia (19% vs. 0%), hypogeusia (32% vs. 12%), anosmia (13% vs. 0%), or hyposmia (29% vs. 9%). Significantly lower saliva secretion rates (ml min^?1) were observed in patients with pSS for stimulated (0.62 ± 0.40 vs. 1.57 ± 0.71) and unstimulated (0.08 ± 0.07 vs. 0.29 ± 0.17) saliva. The mean OHIP‐14 score was significantly higher in patients with pSS (16.2 ± 10.8 vs. 2.7 ± 3.1) and was positively correlated with dysgeusia, BST, and halitosis. In conclusion, patients with pSS reported higher occurrence of dysgeusia, BST, and halitosis, and demonstrated relatively impaired chemosensory and salivary functions. The patients’ poorer OHRQoL was associated with dysgeusia, BST, and halitosis.