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- Leipala, JA, et al.
(författare)
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Haemodynamic effects of erythrocyte transfusion in preterm infants
- 2004
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Ingår i: European Journal of Pediatrics. - : Springer Science and Business Media LLC. - 1432-1076 .- 0340-6199. ; 163:7, s. 390-394
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Tidskriftsartikel (refereegranskat)abstract
- The aim of the study was to assess the short-term cardiorespiratory effects of a standard red cell transfusion in very low birth weight (<1500 g) infants undergoing intensive care. A total of 37 infants (birth weight 920+/-230 g, gestational age 27.8+/-2.1 weeks, age at study 6.1+/-3.9 days) with indwelling arterial lines were studied when 10 ml/kg of packed donor red cells were transfused based on clinical judgment. Infants with patent ductus arteriosus and/or inotropic treatment were excluded from the study. Oxygen saturation, left ventricular output, stroke volume, systolic, diastolic and mean arterial pressure, heart rate, and capillary refill time were assessed immediately prior to the transfusion and within an hour after the transfusion was completed. Capillary refill time after the transfusion was significantly shorter than prior to the transfusion (2.1+/-0.9 versus 2.4+/-1.0 s, P =0.033). Left ventricular output, stroke volume and arterial pressures remained unaltered. Oxygen saturation after the transfusion was lower than before the transfusion (94.0+/-3.8 versus 95.3+/-2.5%, P =0.014) despite unaltered oxygen supply. Conclusion: the data suggest that although a red cell transfusion of 10 ml/kg may marginally improve peripheral perfusion, it does not influence cardiac output and arterial blood pressure in normotensive preterm infants. It may, however, cause a transient decrease in oxygen saturation.
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| 3. |
- Ries, M, et al.
(författare)
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The early clinical phenotype of Fabry disease: a study on 35 European children and adolescents.
- 2003
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Ingår i: European Journal of Pediatrics. - 0340-6199. ; 162:11, s. 767-72
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Tidskriftsartikel (refereegranskat)abstract
- Fabry disease (FD) is a debilitating progressive multisystem X-linked lysosomal storage disorder. It was generally believed that the disease affects only adult males. Through systematic pedigree analysis, we identified 35 paediatric FD patients (age 1 to 21 years, mean 12.6 years) in 25 families. Predominant signs in this cohort were: acroparesthesia, hypohidrosis, and cornea verticillata. Neurological and psychological changes, such as tinnitus, recurrent vertigo, headache, diminished level of activity, fatigue, and depression were often observed. Angiokeratoma and gastrointestinal symptoms were frequent. Some patients also showed cardiac abnormalities. Six children and adolescents (three males and three females) over 14 years of age had renal involvement (all with proteinuria, one male had a decreased creatinine clearance of 62 ml/min). No males, but three females (1.5, 4 and 9 years of age), were free of signs and symptoms. Males (n=15, age 1 to 21 years, mean 12.4 years) and females (n=20, age 1.5 to 20 years, mean 12.7 years) showed comparable disease severity. However, the clinical courses demonstrated a wide intra- and interfamilial variability and tended to be more heterogeneous in the girls. Female patients are frequently affected at an early age, not much differently than males. They should be carefully examined because most carriers are symptomatic. CONCLUSION: Fabry disease usually becomes clinically manifest in childhood. Renal involvement can begin in adolescence. The diagnosis is made following a high level of suspicion or systematic pedigree analysis. It is crucial for paediatric Fabry disease patients to have early access to optimal supportive symptomatic management. Enzyme replacement therapy has shown promising effectiveness in adults. Considering its widespread therapeutic and potential preventive benefits, enzyme replacement therapy should be initiated at an early stage, prior to the onset of irreversible complications.
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- Wikkelsö, Carsten, et al.
(författare)
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Subjective visual vertical and Romberg's test correlations in hydrocephalus.
- 2003
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Ingår i: Journal of neurology. - : Springer Science and Business Media LLC. - 0340-5354 .- 1432-1459. ; 250:6, s. 741-5
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Tidskriftsartikel (refereegranskat)abstract
- Exploration of the subjective visual vertical in pitch and Romberg's test in patients with hydrocephalus. Patients Eleven patients with communicating normal pressure hydrocephalus and six with non-communicating hydrocephalus due to aqueductal stenosis were examined before and after surgical treatment.The subjective visual vertical was examined with a simple, custom-made articulated rod. The rod was faintly illuminated from within so as to appear floating in space when presented in darkness. The test task was to manually adjust the rod's position in pitch so that it appeared perfectly vertical. Romberg's test was performed under standardized conditions.Patients with a backward movement on Romberg's test showed a deviation of the subjective visual vertical, tilting the upper end of the rod closer to their eyes. Patients moving in other directions tilted the rod in the opposite direction (p < 0.01). The time in which the patients managed Romberg's test with open eyes correlated with the intratest variability of rod placement (r = 0.86, p < 0.001). After surgery, improvements on Romberg's test correlated with a decreased intratest variability of the subjective visual vertical. There were no differences between the two patient groups.Quantitative measurements of the subjective visual vertical in pitch correlate strongly with results of Romberg's test in patients with hydrocephalus.
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