We analyzed the characteristics of young infants diagnosed with vitamin D deficiency in early infancy at 2 medical centers in Turkey. In this retrospective, cross-sectional study, the clinical, biochemical, and radiographic findings of infants who were diagnosed with vitamin D deficiency at <3 mo of age between May 2001 and May 2003 were reviewed. A total of 42 infants (27 boys and 15 girls) were diagnosed with vitamin D deficiency in the first 3 mo of life during this 2-y period. The age of infants at diagnosis was 60 +/- 19 d (range 32-112 d). The majority (78.7%) presented with seizures. No skeletal deformities were detected clinically, and radiological findings were subtle. All infants had low serum calcium levels but serum phosphorous levels varied. Eight infants (19.0%) had low, 19 (45.3%) had normal, and 15 (35.7%) had elevated serum phosphorous levels. Serum 25-hydroxyvitamin D levels in those measured (29 infants and 15 mothers) were <37.5 nmol/L. Most infants (83%) were exclusively breast-fed without supplemental vitamin D, and none of the mothers were supplemented with vitamin D during pregnancy. All mothers had limited sunlight exposure and 33 of 42 mothers (78.6%) wore concealing clothing. The majority of young infants diagnosed with vitamin D deficiency present with seizures, have low dietary vitamin D intake, and mothers with poor vitamin D reserves. Evaluation of vitamin D status should be included into the workup of hypocalcemia in early infancy. Prevention of deficiency by supplementing pregnant women and infants who are exclusively breast-fed is essential.
Lung transplantation is the only viable option for patients suffering from otherwise incurable end-stage pulmonary diseases such as chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis. Despite aggressive immunosuppression, acute rejection of the lung allograft occurs in over half of transplant recipients, and the factors that promote lung acceptance are poorly understood. The contribution of lymphatic vessels to transplant pathophysiology remains controversial, and data that directly address the exact roles of lymphatic vessels in lung allograft function and survival are limited. Here, we have shown that there is a marked decline in the density of lymphatic vessels, accompanied by accumulation of low-MW hyaluronan (HA) in mouse orthotopic allografts undergoing rejection. We found that stimulation of lymphangiogenesis with VEGF-C156S, a mutant form of VEGF-C with selective VEGFR-3 binding, alleviates an established rejection response and improves clearance of HA from the lung allograft. Longitudinal analysis of transbronchial biopsies from human lung transplant recipients demonstrated an association between resolution of acute lung rejection and decreased HA in the graft tissue. Taken together, these results indicate that lymphatic vessel formation after lung transplantation mediates HA drainage and suggest that treatments to stimulate lymphangiogenesis have promise for improving graft outcomes.
Pediatric lung transplantation is a lifesaving option for patients with end stage lung disease, although the scarcity of suitable donor organs results in long wait times and increased waitlist mortality. Many pediatric centers consider mechanical ventilatory support, such as long-term invasive ventilation and ECMO, a contraindication to lung transplantation. We hypothesized that current survival rates and outcomes for patients on mechanical ventilatory support in the pre-transplant period were not remarkably different. In our retrospective analysis we included patients between the ages of 0-21 years listed for lung transplantation from deceased donors between 2007 and 2014 at our institution. One-year survival outcomes were compared between three groups of patients: (i) patients bridged to transplant on ECMO (n = 6, 1-year survival = 67%); (ii) patients needing mechanical ventilation (either through endotracheal intubation or tracheostomy) but not ECMO (n = 12, 1-year survival = 75%); and (iii) patients who did not need endotracheal ventilation, tracheostomy, or ECMO (n = 25, 1-year survival = 88%). Comparison of outcomes of transplanted patients between these three groups were not statistically different in terms of successful hospital discharge and 1-year survival rates (P > 0.05). We believe that "bridging" the end-stage lung disease patient with long-term mechanical ventilation and/or ECMO support is a reasonable option in selected patients until suitable donors become available. Pediatr Pulmonol. 2017;52:360-366. © 2016 Wiley Periodicals, Inc.
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