Effects of inhaled corticosteroids and inhaled cromolyn sodium on urinary growth hormone excretion in asthmatic children

Over the past few years there has been an increasing awareness that asthma is a chronic inflammatory airways disease. The current therapeutic strategies for treating asthma focus on suppressing the inflammatory process by using cromones or inhaled corticosteroids (ICS). The beneficial effects of ICS in asthma are now well known, but its detrimental effect on linear growth remains a controversial issue. The aim of this open label, nonrandomized, cross-sectional, one-time study was to determine the influence of these drugs on urinary growth hormone (U-GH) levels in prepubertal asthmatic children. U-GH levels were measured in 47 prepubertal asthmatic children who had been treated for at least 6 months with either ICS (beclomethasone or budesonide at a mean daily dose of 360 μg) or with 80 mg daily dose of cromolyn sodium (CrS). There were also nine healthy children who served as a control. These three groups of children were matched for age and gender ratio. The mean level of U-GH in the CrS-treated group was 2.94 ± 0.96 ng/night; this was significantly higher compared to the mean level of the ICS-treated group (1.99 ± 0.83 ng/night; P < 0.001) and to the mean level of the control group (1.98 ± 0.39 ng/night; P < 0.006). There was no significant difference between the mean level of U-GH in the group treated by ICS and the controls (P < 0.9). These results show that the mean levels of U-GH secretion of the children who were treated by CrS for 6 months was significantly increased, compared to the mean U-GH level of the ICS-treated group and the controls. The mean U-GH levels in the last two groups showed no statistically significant difference. Pediatr Pulmonol. 1998; 26:339–343. © 1998 Wiley-Liss, Inc.     

Cancer care at the hospital–community interface: Perspectives of patients from different cultural and ethnic groups

Objective

To examine perceptions of cancer patients from different cultural and ethnic groups regarding the quality of their care at the hospital–community interface.

Methods

Cross-sectional study of cancer patients from a large tertiary center in Israel. Patients were surveyed on the quality of their primary-care and on their transition from the hospital. Regression analyses were used to examine differences among Hebrew-, Russian-, and Arabic-speaking patients in their assessment of six primary-care domains and of their care transition process.

Results

422 patients completed the survey. Russian speakers gave the lowest and Arabic speakers the highest ratings for all primary care domains. Arabic speakers also gave higher ratings for their care transition process than the other two groups. Minority patients’ primary-care physicians were significantly more likely than Hebrew speakers’ physicians to facilitate the hospital transition process by reviewing the discharge recommendations.

Conclusions

Cancer patients from different ethnic groups differ in their primary-care experience and their perceptions of their hospital to community transition. Primary-care physicians serve as facilitators of care transitions by discussing discharge recommendations with their minority patients.

Practice implications

Reviewing the discharge recommendations with the patient at the post-discharge primary-care visit is an important contributor to high quality transitional care.     

Clinical and laboratory characteristics of adolescents with both polycystic ovary disease and anorexia nervosa

In girls evaluated for anorexia nervosa (AN), coexistence of polycystic ovary disease (PCOS) was found when menstrual irregularities existed before weight loss, cessation of menses occurred at a relatively elevated body mass index, and LH and estrogen levels were elevated compared with adolescents with AN alone. In adolescents with PCOS + AN weight gain beyond recommended weight should not be advised.     

Amniotic trisomy 11 mosaicism--is it a benign finding?

OBJECTIVES: A case of prenatally diagnosed trisomy 11 mosaicism with a normal outcome is reported and the medical literature on prenatal detection of this finding is reviewed. METHODS: Proportion of cells with trisomy 11 was evaluated in amniocytes, fetal blood lymphocytes, newborn fibroblasts and urinary epithelial cells. Karyotype studies and fluorescence in situ hybridization analysis using the 11q13LS1 CCND1 probe were performed. RESULTS: Trisomy 11 level III mosaicism of 26% was detected in amniotic fluid cells. Periumbilical blood sampling showed a normal fetal karyotype. No fetal structural abnormalities were noted on ultrasound scan. The infant was spontaneously delivered and had normal physical findings at birth. No evidence of trisomic cells was found on extensive postnatal evaluation, implying an extraembryonic origin. Molecular analysis excluded uniparental disomy of chromosome 11. At 1 year of age, the baby is developing normally. CONCLUSIONS: Only three reports on trisomy 11 mosaicism identified at amniocentesis have been published previously, all with a normal outcome. Additional cases of prenatally diagnosed mosaicism for trisomy 11 are necessary to assess more accurately the clinical significance of this finding.     

Peripheral B cell receptor editing may promote the production of high-affinity autoantibodies in CD22-deficient mice

CD22-deficient mice are characterized by B cell hyperactivity and autoimmunity. We have constructed knock-in CD22-/- mice, expressing an anti-DNA heavy (H) chain (D42), alone or combined with Vkappa1-Jkappa1 or Vkappa8-Jkappa5 light (L) chains. The Ig-targeted mice produced a lupus-like serology that was age- and sex-dependent. High-affinity IgG autoantibodies were largely dependent on the selection of B cells with a particular H/L combination, in which a non-transgenic, endogenous L chain was assembled by secondary rearrangements through the mechanism of receptor editing. Moreover, we present evidence that these secondary rearrangements are very prominent in splenic peripheral B cells. Since CD22 is primarily expressed on the surface of peripheral B cells, we propose a model for the development of a lupus-like autoimmune disease by a combination of peripheral receptor editing and abnormal B cell activation.     

Schimke immuno-osseous dysplasia: expression of SMARCAL1 in blood and kidney provides novel insight into disease phenotype

Schimke immuno-osseous dysplasia (SIOD) is an autosomal recessive disorder caused by loss-of-function mutations in SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily a-like 1 (SMARCAL1), with clinical features of growth retardation, spondylo-epiphyseal dysplasia, nephrotic syndrome, and immunodeficiency. We report a patient with SIOD and SMARCAL1 splice mutation (IVS4-2 A>G) in a nonconsanguineous Ashkenazi family, who came to our attention at 1 mo of age due to renal malformation and only later developed signs compatible with Schimke. Interestingly, residual SMARCAL1 mRNA levels in the patient's peripheral blood were lower compared with those observed in both asymptomatic brothers' carrying the same bi-allelic mutation, whereas the latter had levels similar to those found in heterozygous carriers (parents and sister). Examination of the carrier frequency of the splice mutation in the Ashkenazi population demonstrated 1 carrier in 760 DNA samples. In situ localization of SMARCAL1 in human kidneys as well as analysis of its temporal expression during murine nephrogenesis and in the metanephric organ culture suggested a role in the early renal progenitor population and after renal maturation. Thus, disease severity within the same family might be modified by the splicing machinery. The renal expression pattern of SMARCAL1 explains a broader spectrum of renal disease in SIOD than previously described.     

Predictive value of amplitude-integrated electroencephalography pattern and voltage in asphyxiated term infants

The aim of this study was to correlate amplitude-integrated electroencephalography soon after birth with neurodevelopmental outcome in children who suffered from hypoxic-ischemic encephalopathy. Near term infants with hypoxic-ischemic encephalopathy and amplitude-integrated electroencephalography recording before 6 hours of age were included. Neurologic and cognitive outcome were assessed at 30 months of age and over. Outcome was correlated with either the pattern or voltage of the tracing. Thirty-nine infants were included. Eight died in the immediate neonatal period. At the age of 3 and 6 hours, sensitivity of low voltage to poor outcome was 33% and 42% respectively and of burst suppression pattern to poor outcome was 83% and 75% respectively. Association of voltage to outcome was significant only at 6 hours of age (P = 0.025). Association of pattern to outcome was significant both at 3 and 6 hours of age (P = 0.003, 0.008). These data on amplitude-integrated electroencephalography predictive value early in life were similar to previous studies. Burst suppression pattern, as early as 3 hours of age, is associated with poor outcome. At the age of 6 hours, both low voltage and burst suppression are associated with poor outcome. Pattern seems more sensitive than voltage.