No abstract

For elementary school children with atopic dermatitis, a skin care program using shower therapy was performed during the school lunch break for 6 weeks from June to July in 2004 and 2005. All 53 participants showed an improvement in their atopic dermatitis during the 6-week periods studied. Skin care with daily showering at an elementary school was thus found to be effective for the treatment of atopic dermatitis.

Our data highlight the characteristics of breath sounds in infants with risk factors for asthma. The breath sound analysis may be useful for assessing the airways of infants for asthma development.

As antenatal environment may influence the development of atopy-predisposing immune response, cord blood cytokine productions may be an important predictor for wheezing. We investigated cord blood cytokines in a prospective birth cohort, intensively monitored for wheezy infant outcome at 1 yr. Cord blood serum samples from 269 children were assayed for interleukin (IL)-1beta, -2, -4 to -8, -10, -12 (p70), -13, and -17, interferon-gamma, tumor necrosis factor-alpha, granulocyte-macrophage colony-stimulating factor, granulocyte colony-stimulating factor (G-CSF), monocyte chemotactic protein-1, and macrophage inflammatory protein-1beta. Associations between family histories, antenatal and perinatal factors, cord blood cytokine concentrations, and wheezy infant outcomes (wheezing more than two times) were analyzed. In cord blood sera from 269 children, there were associations between high levels of IL-6, -8 and G-CSF concentrations, and cesarean section. Data at 1 yr were obtained from 213 infants, including 33 wheezy infants. Risk of wheezing was related to gestational age, birth weight, cesarean section, and maternal eczema, but not to bacterial/viral infection during pregnancy, maternal asthma, maternal smoking, or paternal history. High level of cord blood IL-8 concentration had a significant association with wheezy infant outcomes at 1 yr (p = 0.025). By using multivariate logistic regression analysis, birth weight [odds ratio(OR) = 0.998, 95% confidence interval (CI) = 0.997-1.000] and maternal eczema (OR = 5.356, 95% CI = 1.340-21.41), but no other factors, were significant predictors of wheezy infants. Birth weight, gestational age, and maternal history were important risk factors for wheezing in the first year of life. Several cord blood cytokine productions were influenced by cesarean section, and IL-8 may be a predictor for recurrent wheezing at 1 yr.

Interstitial 1q deletions have been classified into three groups: proximal deletion (1q21-22q25), intermediate (1q24-25q32), and distal (1q42-43qter) [Taysi et al., 1982]. To our knowledge, only seven deletions have been characterized by molecular methods [Franco et al., 1991;Takano et al., 1997;Melis et al., 1998;Pallotta et al., 2001;Hoglund et al., 2003;Chaabouni et al., 2006;Descartes et al., 2008]. We report on a patient with 1q24.3-q31.2 deletion, which was thoroughly analyzed by high density SNP array as well as junctional cloning.The propositus is a 3 1 = 3 -year-old boy. He was born at 35 weeks of gestation by cesarean due to fetal distress of nonconsanguineous 39-year-old mother and 37-year-old father. Intrauterine growth retardation was suspected at 27 weeks of gestation. Birth length was 36 cm (À3.7 SD), weight 1,324 g (À3.8 SD), and head circumference (OFC) 29 cm (À1.5 SD). At age of 3 1 = 3 years, his length is 65 cm (À8.5 SD), weight 5,890 g (À4.5 SD), and OFC 41.6 cm (À5.5 SD), indicating obvious pre-and postnatal growth retardation. Multiple anomalies were recognized: prominent forehead, sparse, and fine hair, decreased expression of the face, deep-set eyes, prominent cupid bow, left ptosis, left exotropia and strabismus, short philtrum, downturned corners of the mouth, high-arched palate, lowset ears, small hands and feet, bilateral simian creases, bilateral fifth clinodactyly, bilateral inguinal hernia, cryptorchidism, and small penis.Radiographs showed the following findings. The upper thorax is asymmetrically narrow as a result of unequally short upper ribs. The ribs were 12 in number. Incomplete congruence of the hip joints was noted together with deep acetabular angles. The distal ulnae were relatively elongated, and they were somewhat displaced ulnarly. The distal radial articular surfaces were unusually tilted radially. In the hands, the middle phalanges, particularly of the index and little fingers, were short and bullet-shaped. The terminal tufts of the distal phalanges, particularly of the second, were somewhat hypoplastic. The fifth metacarpals were broad and short. Carpal ossification was significantly delayed. In feet, he middle and distal phanageal ossification was incomplete.Bilateral low-tone hearing loss (mixed-type) and otitis media were observed. He was barely able to walk at 3 years. Head magnetic resonance imaging showed no abnormality. Serum TSH, fT3, and fT4 were normal. IGF-1 and IGFBP-3 were low [4.0 ng/ml (normal range: 11-172 ng/ml) and 0.34 mg/ml (normal range: 1.02-2.50 mg/ml), respectively] and GH stimulation test by clonidine and L-DOPA indicated GH deficiency [0.76 ng/ml and 0.49 ng/ml at maximum, respectively (pathological range: <6 ng/ml)]. Serum antithrombin III (AT III) was also at a low level [13.7 mg/dl (normal range: 23.6-33.5 mg/dl), 48% in activity (normal range: 79-121%)].GTG-binding chromosome analysis of the patient's blood lymphocytes demonstrated 46,XY,del(1)(q23q25),t(4;11)(q31.3;q21) (Fig. 1a).

No abstract

Systemic-onset juvenile idiopathic arthritis (s-JIA) is a rare inflammatory disease classified as a subtype of chronic childhood arthritis, manifested by spiking fever, erythematous skin rash, pericarditis and hepatosplenomegaly. The genetic background underlying s-JIA remains poorly understood. To detect disease-related copy number variations (CNVs), we performed singlenucleotide polymorphism array analysis in 50 patients with s-JIA. We detected many CNVs, but most of them were inherited from either of normal-phenotype parents. However, in one patient, we could identify two de novo microduplications at 19q13.42 with the size of 77 and 622 kb, separated by a 109-kb segment of normal copy number. The duplications encompass NLRP family (NLRP2, NLRP9 and NLRP11) as well as IL11 and HSPBP1, all of which have an important role in inflammatory pathways. These genes may significantly contribute to the pathogenesis of s-JIA.

Lung fibroblasts are a major source of several cytokines including CC chemokine eotaxin. We aimed to study the regulation of eotaxin-1/CCL11 production by dexamethasone and analyze its molecular mechanisms in human lung fibroblasts. Normal human lung fibroblast cells were exposed to IL-4 (40 ng/ml) and/or dexamethasone (10(-6)-10(-9) M), and eotaxin mRNA expression and production was evaluated. Mechanisms of transcriptional regulation were assessed by Western blotting and dual luciferase assay for eotaxin promoter. The effects of dexamethasone on suppressor of cytokine signaling (SOCS)-1 and eotaxin mRNA expression in the cells transfected with expression vector (pAcGFP1-C1) or short interfering RNA (siRNA) for SOCS-1 were also investigated. Within 24 hours, dexamethasone inhibited IL-4-induced eotaxin mRNA expression and protein production, while eotaxin production was markedly increased at 48 and 72 hours after coincubation with IL-4 and dexamethasone. IL-4-induced eotaxin promoter activity was inhibited by dexamethasone at 8 hours, but enhanced at 48 hours after coincubation. Dexamethasone suppressed SOCS-1 mRNA expression but enhanced IL-4-induced STAT6 phosphorylation at 36 to 48 hours after coincubation. Enhanced expression of eotaxin mRNA by dexamethasone 48 hours after coincubation was completely diminished in the cells transfected with either expression vector or siRNA for SOCS-1. These results indicated that dexamethasone, depending on the exposure duration, can either inhibit or enhance IL-4-induced expression and production of eotaxin in the lung fibroblasts. The mechanisms of later enhanced production may depend on the prolonged transcriptional activity of the eotaxin gene, in part due to inhibition of SOCS-1 expression.