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Article: Transition of young adults with phenylketonuria from pediatric to adult care.
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ABSTRACT: Transition from pediatric to adult health care is a particularly vulnerable period for patients with inborn metabolic diseases. Aim of the present study was to evaluate the current transition situation of patients with phenylketonuria (PKU) in Leipzig, Germany, by analysis of the medical care, metabolic control, patients' satisfaction, socio-economic and psychosocial status, in order to identify areas of weakness and potential improvement. Patients who had been transferred from pediatric to adult medical care between 2005 and 2008 were identified. An interview was performed using a questionnaire. Pediatric case notes and the present physician's case notes were analyzed retrospectively. Socio-demographic data were compared to data derived from the annual statistics of the city of Leipzig, Germany in 2008. seventy two transferred patients were identified and included in the study, 48 patients responded to the questionnaire, the data of 24 non-responders were analysed retrospectively. About 90% of the responding patients with PKU were satisfied with the current transition situation. However, they agreed to several suggestions of improvement. Most specifically an interdisciplinary appointment before the definite transfer to the adult clinics was asked for. At the time of transition, most of the patients were in good metabolic control according to current treatment guidelines (median dried blood phenylalanine concentration 853 μmol/l before versus 690 μmol/l after transition). Of the interviewed patients 92% were still on a low phenylalanine diet in combination with the intake of a phenylalanine free amino acid mixture. Of the interviewees 77% carried a secondary school certificate or a secondary modern school qualification, but only 19% had achieved senior high school diploma (controls 38.2%). Marital status was comparable with the population of Leipzig. However, fewer patients with PKU had children (15% versus 37%). Transition of patients with PKU from pediatric to adult care seems to be successful in Leipzig. Patients were mostly satisfied with the transition situation. Still, some suggestions for improvements appeared to be desirable. During transition medical care and metabolic control were stable. However, with regard to psychosocial and socioeconomic data differences to the control population were detected.Journal of Inherited Metabolic Disease 02/2011; 34(3):701-9. · 3.58 Impact Factor -
Article: Autonomic dysfunction in rheumatic diseases.
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ABSTRACT: Patients who have rheumatic diseases often present with dysfunctions that are related to the autonomic nervous system (ANS) and are due to peripheral autonomic neuropathy or central changes. This article describes the prevalence of autonomic dysfunctions in patients who have rheumatic diseases. In the second part of this article, another form of ANS dysfunction-complex regional pain syndromes-is demonstrated. Clinically, these syndromes are characterized by pain (spontaneous, hyperalgesia, allodynia); active movement disorders, including an increased physiologic tremor, abnormal regulation of blood flow and sweating, edema of skin and subcutaneous tissues; and trophic changes of skin, appendages of skin, and subcutaneous tissues. In conclusion, this discussion shows that alterations of the ANS occur in rheumatic and related diseases, that these alterations may be involved in the pathogenesis of these diseases, and that we need more refined methods to study the changes that are related to the ANS.Rheumatic Disease Clinics of North America 03/2005; 31(1):61-75, viii. · 3.02 Impact Factor -
Article: Chronic urticaria and angioedema with concomitant eosinophilic vasculitis due to Trichinella infection.
Acta Dermato Venereologica 02/2008; 88(1):78-9. · 3.18 Impact Factor -
Article: Regulation of beta2-adrenergic receptors on CD4 and CD8 positive lymphocytes by cytokines in vitro.
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ABSTRACT: Increasing evidence points to a close relationship between the autonomic nervous system and the immune system. To further investigate mechanisms regulating beta2-adrenergic receptor (beta2R) expression in lymphocytes, the influence of cytokines on the density of beta2R on purified CD4+ and CD8+ lymphocytes was determined in vitro. beta2R were determined by means of a radioligand binding assay with (125I)iodocyanopindolol. CD4+ and CD8+ lymphocytes were incubated with catecholamines, interleukin 1beta (IL-1beta) and interleukin 2 (IL-2) for 6-72 h. The results demonstrate declining beta2R numbers on CD4+ and CD8+ lymphocytes in vitro augmented by epinephrine. IL-1beta has no effect on beta2R expression compared to medium. However, incubation with IL-2 resulted in an up-regulation of beta2R on CD8+ lymphocytes. Thus, the study demonstrates a differential regulation of beta2R on T-lymphocyte subpopulations with CD8+ lymphocytes being more susceptible to mechanisms of beta2R modulation than CD4+ lymphocytes. The findings further strengthen the concept of a close interplay between the autonomic nervous system and the immune system.Cytokine 01/2002; 16(6):205-9. · 3.02 Impact Factor -
Article: Norepinephrine inhibits energy metabolism of human peripheral blood mononuclear cells via adrenergic receptors.
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ABSTRACT: Previous studies demonstrated that the adaptive response to stressors and inflammatory signals involves the activation of the autonomous [corrected] nervous system. Catecholamines have been shown to modulate the activity of various immune effector cells directly via membrane adrenergic receptors. Here, we investigated immediate effects of norepinephrine on energy metabolism of immune cells. Norepinephrine inhibits oxygen consumption of human peripheral blood mononuclear cells at concentrations that are relevant to its physiological range. The beta-adrenoreceptor antagonist propranolol, but not the alpha-adrenoreceptor antagonist phentolamine reversed the norepinephrine induced inhibition in quiescent cells. Conversely, phentolamine but not propranolol is capable of blocking norepinephrine mediated effects in mitogen activated human peripheral blood mononuclear cells. Our data indicate that the sensitization of alpha- and beta-adrenoreceptors on immune cells is differentially regulated, and that these processes depend on the activation state of these cells. These findings have important implications for the understanding of stress-induced suppression of immune function and may contribute to the elucidation of the pathogenesis of immunologically mediated diseases.Bioscience Reports 11/2001; 21(5):627-35. · 2.38 Impact Factor
