Upper airway features of unilateral cleft lip and palate patients in different growth stages

Objectives:To compare growth-related changes of skeletal and upper airway features of unilateral cleft lip and palate subjects (UCLP) with non-cleft control (NCC) subjects by using lateral cephalograms.Materials and Methods:The sample comprised 238 subjects, collected cross-sectionally, divided into 2 groups: 94 with UCLP, and 144 NCC, subdivided into 4 groups according to their growth stages by using cervical vertebral maturation stage (CVMS). The subgroups were defined as early childhood (stage 1), prepubertal (stage 2: CVMS I and II), pubertal (stage 3: CVMS III and IV), and postpubertal (stage 4: CVMS V and VI).Results:The maxilla was more retrognathic at stages 2, 3, and 4 in females with UCLP. The mandible was more retrognathic in UCLP at stage 1 in males, and stages 2 and 3 in females. ANB (angle between NA plane and NB plane) was significantly smaller in UCLP subjects at stage 4 for both sexes. A vertical growth pattern was seen in UCLP subjects except males at stages 2 and 3, and females at stage 2. Posterior airway space was significantly narrower at all stages in males and after stage 1 in females. Middle airway space was significantly wider at all stages in females and after stage 1 in males. Epiglottic airway space was significantly narrower in males at stage 3.Conclusions:Age- and sex-dependent differences in skeletal morphology and upper-airway widths of the UCLP subjects were identified when compared with controls.     

The effects of iron on FGF23-mediated Ca–P metabolism in CKD patients

Background

Fibroblast growth factor 23 (FGF23) is an important counterregulatory hormone for phosphate homeostasis. Since it has been reported that iron administration induces hypophosphatemic osteomalacia by triggering FGF23 synthesis, we hypothesized that iron administration might lead to a further increase in FGF23, resulting in alterations to Ca–P metabolism in a stage 5 CKD population.

Methods

This cross-sectional study was performed in a single center, and involved 73 hemodialysis patients (47.7 ± 15.74 years old, 68.5 % men), 29 peritoneal dialysis patients (44.55 ± 15.05 years old, 62.1 % men), and 55 healthy (43.57 ± 14.36 years old, 55.6 % men) subjects. The dialysis group was subcategorized according to iron therapy administration into users and nonusers.

Results

The median iFGF23 level was significantly higher in the dialysis population than in the healthy controls [88.050 (25.2–1038.3) pg/ml versus 46.95 (2.4–356) pg/ml (p < 0.001)]. In the dialysis population, a significantly lower median iFGF23 level was observed in iron therapy users than in nonusers [87.6 (25.2–1038.3) versus 119 (51.6–1031); respectively, p = 0.045]. A significant negative association between iron administration and iFGF23 level was revealed by both univariate (r = ?0.237, p = 0.016) and multivariate (β = ?0.221, p = 0.032) analysis. No association was found between iFGF23 and serum ferritin and iron levels. Also, there was no association between iron therapy and serum phosphate level.

Conclusion

In contrast to what is seen for the general population, this study showed that there was a negative relationship between iron administration and serum iFGF23 level in a dialysis population. We can therefore conclude that if high levels of FGF23 are harmful, iron therapy may have a beneficial effect on bone metabolism by reducing FGF23 levels in a dialysis population.     

Elevated TRIB2 with NOTCH1 activation in paediatric/adult T-ALL

TRIB2 is a potent oncogene, elevated in a subset of human acute myeloid leukaemias (AML) with a mixed myeloid/lymphoid phenotype and NOTCH1 mutations. Although rare in AML, activating NOTCH1 mutations occur in 50% of all T cell acute lymphoblastic leukaemias (T-ALL). TRIB2 is a NOTCH1 target gene that functions in the degradation of key proteins and modulation of MAPK signalling pathways, implicated in haematopoietic cell survival and proliferation. This study showed that TRIB2 expression level is highest in the lymphoid compartment of normal haematopoietic cells, specifically in T cells. Analysis of TRIB2 expression across 16 different subtypes of human leukaemia demonstrated that TRIB2 expression was higher in ALL phenotypes versus all other phenotypes including AML, chronic lymphocytic leukaemia (CLL), myelodysplastic syndrome (MDS) and chronic myeloid leukaemia (CML). A T cell profile was distinguished by high TRIB2 expression in normal and malignant haematopoiesis. High TRIB2 expression was seen in T-ALL with normal karyotype and correlated with NOTCH signalling pathways. High TRIB2 expression correlated with NOTCH1/FBXW7 mutations in a paediatric T-ALL cohort, strongly linking NOTCH1 activation and high TRIB2 expression in paediatric T-ALL. The relationship between TRIB2 and T cell signalling pathways uniquely identifies leukaemia subtypes and will be useful in the advancement of our understanding of T cell and ALL biology.     

Effect of 1α-25-dihydroxyvitamin D3 on intimal hyperplasia developing in vascular anastomoses: a rabbit model

Introduction

A common problem encountered in routine daily practice of cardiovascular surgery is migration of smooth muscle cells leading to intimal hyperplasia developing at vascular anastomosis sites which then causes luminal narrowing. The aim of this study was to investigate the antiproliferative effect of 1,25 (OH)₂D₃ on intimal hyperplasia.

Material and methods

Twenty-one male white New Zealand rabbits weighing 2-3 kg were selected. There were 3 groups of animals each consisting of 7 rabbits. Group 1 was the control group. Group 2 was the sham group and group 3 consisted of rabbits receiving 1,25 (OH)₂D₃. The right carotid arteries of the subjects in groups 2 and 3 were transected and re-anastomosed. A daily dose of 25 ng 1,25 (OH)₂D₃ per 100 g body weight was administered for 14 days to rabbits in group 3. Rabbits in group 2 were not subject to any pharmaceutical agent. All the subjects were sacrificed at the end of the 28^th postoperative day. Their right carotid arteries were resected and then investigated histopathologically.

Results

Intimal thickness and intimal area were measured as significantly lower in group 1 when compared with the other groups (p = 0.004). In group 3, the ratios of thickness of tunica intima/thickness of tunica media and area of tunica intima/area of tunica media were significantly lower than those of group 2 (p = 0.015, p = 0.003).

Conclusions

1,25 (OH)₂D₃, the active metabolite of vitamin D, reduces the intimal hyperplasia developing after vascular anastomoses.     

Induced sputum as a method for detection of systemic sclerosis-related interstitial lung disease

Inducted sputum (IS) is a non-invasive procedure that can be used for collection of airway secretions. The aim of our study is to evaluate the clinical usefulness of IS for detection of airway inflammation in systemic sclerosis (SSc). Bronchoalveolar lavage and IS were performed to 20 patients with SSc. Eighteen patients who were referred to pulmonary medicine for bronchoalveolar lavage due to other reasons were also recruited for cell counts comparisons. Spirometry, echocardiography and thorax CT (HRCT) imaging were also performed to all patients. Mean macrophage and lymphocyte counts were found to be increased in IS of SSc patients compared with that of control (58.4?±?14.5% vs. 31.3?±?16.3%, 30.2?±?15.4% vs. 15.0?±?11.5% P?<?0.001), whereas mean neutrophil count was lower in the SSc patients (4.1?±?4.5% vs. 17.2?±?13.1%, P?<?0.05). Significant correlations were noted between BAL and IS findings for macrophage (r?=?0.55, P?=?0.02) lymphocyte (r?=?0.65, P?<?0.01) and total cell counts (r?=?0.45, P?=?0.06). IS is an easy and reliable method for the detection of alveolitis and can be used for early detection of lung involvement in scleroderma.