Matthias Henschen

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Griscelli syndrome (GS) was diagnosed in a 2-year-old patient with oculocutaneous albinism and immunodeficiency, but sequencing of RAB27a revealed only a heterozygous mutation. Due to impaired natural killer (NK) and T-cell cytotoxicity implying a high risk of developing hemophagocytic lymphohistiocytosis (HLH), he was prepared for hematopoietic stem cell(More)
OBJECTIVE To assess airway function at 1 year and compare this with similar measurements made shortly after birth in preterm infants without clinical neonatal respiratory disease. STUDY DESIGN Infants born at </=36 weeks' gestational age were eligible if they required no neonatal ventilatory support and were otherwise healthy. Paired measurements of(More)
We examined whether the adverse effects of prenatal exposure to tobacco on lung development are limited to the last weeks of gestation by comparing respiratory function in preterm infants whose mothers had and had not smoked during pregnancy. Maximal forced expiratory flow (Vmax FRC) and time to peak tidal expiratory flow as a proportion of total expiratory(More)
The relationship between nitric oxide in exhaled air, levels of sputum eosinophils, sputum eosinophil cationic protein (ECP) and urinary eosinophil protein X (EPX) excretion has not yet been investigated in corticosteroid-dependent childhood asthma. Therefore, taking 25 children with stable asthma (mean age 11.2 yrs) treated with inhaled corticosteroids and(More)
The progress of infant lung function testing has been retarded by both the lack of user-friendly, widely available and affordable equipment and the lack of standardized methodology. The European Respiratory Society/American Thoracic Society Task Force on Standards for Infant Respiratory Function Testing was formed in an attempt to address these(More)
Neonatal hyperthyroidism in the absence of maternal autoimmune thyroid disease and without thyroid-stimulating antibodies in the child is rare. We here describe a boy with severe intrauterine hyperthyroidism and advanced bone age in the absence of thyroid-stimulating autoantibodies. After long term antithyroid treatment and relapse of hyperthyroidism, a(More)
High-frequency respiratory impedance data measured noninvasively by the high-speed interrupter technique (HIT), particularly the first antiresonance frequency (f(ar,1)), is related to airway wall mechanics. The aim of this study was to evaluate the feasibility and repeatability of HIT in unsedated pre-term infants, and to compare values of f(ar,1) from 18(More)
Pediatric reference ranges for osteocalcin measured by a new, fully automated, chemiluminescent immunometric assay on the Immulite immunoanalyzer are presented. Samples from 627 children, ranging from newborns to 18 years of age, were measured. Osteocalcin values are generally higher in children than in adults, highest levels being reached during the(More)
While maximal expiratory flow at functional residual capacity, calculated from partial expiratory flow volume curves (V'maxFRC), is a valuable measure of peripheral airway function in infants, limited data are available in preterm infants despite their high prevalence of respiratory problems. To investigate the influence of gender and ethnic group, V'maxFRC(More)