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Background. Shiga toxin-producing, enteroaggregative Escherichia coli was responsible for the 2011 outbreak of haemolytic uraemic syndrome (HUS). The present single-centre, observational study describes the 1-year course of the disease with an emphasis on kidney function. Outcome data after 1 year are associated with treatment and patient characteristics at onset of HUS. Methods. Patients were treated according to a standardized approach of supportive care, including a limited number of plasmapheresis. On top of this treatment, patients with severe HUS (n = 35) received eculizumab, a humanized anti-C5 monoclonal antibody inhibiting terminal complement activation. The per-protocol decision--to start or omit an extended therapy with eculizumab accompanied by azithromycin--separated the patients into two groups and marked Day 0 of the prospective study. Standardized visits assessed the patients' well-being, kidney function, neurological symptoms, haematological changes and blood pressure. Results. Fifty-six patients were regularly seen during the followup. All patients had survived without end-stage renal disease. Young(er) age alleviated restoring kidney function after acute kidney injury even in severe HUS. After 1 year, kidney function was affected with proteinuria [26.7%; 95% confidence interval (CI) 13.8-39.6], increased serum creatinine (4.4%, CI 0.0-10.4), increased cystatin C (46.7%, CI 32.1-61.3) and reduced (<90 mL/ min) estimated glomerular filtration rate (46.7%, CI 32.1-61.3). Nine of the 36 patients without previous hypertension developed de novo hypertension (25%, CI 10.9-39.1). All these patients had severe HUS. Conclusions. Although shiga toxin-producing Escherichia coli (STEC)-HUS induced by O104:H4 was a life-threatening acute disease, follow-up showed a good recovery of organ function in all patients. Whereas kidney function recovered even after longer duration of dialysis, chronic hypertension developed after severe HUS with neurological symptoms and could not be prevented by the extended therapy. [ABSTRACT FROM AUTHOR]

Hintergrund: Mittels Immunadsorption (IA) können Immunglobuline und Immunkomplexe aus dem Plasma entfernt werden. Während dieses therapeutische Verfahren beim Pemphigus vulgaris bereits etabliert ist, wird es bei anderen blasenbildenden Autoimmundermatosen bislang nur sporadisch eingesetzt. Das bullöse Pemphigoid (BP) spricht zwar meist gut auf eine Therapie mit topischen und systemischen Kortikosteroiden an, jedoch könnte bei Patienten mit ausgedehnten Läsionen oder bei einem Rezidiv die rasche Reduktion der pathogenen Autoantikörper eine effektive adjuvante Therapie darstellen., Patienten Und Methodik: Fallserie mit 20 Patienten (13 Frauen, 7 Männer; mittleres Alter 78,6 Jahre; 56-94 Jahre) mit schwerem oder refraktärem BP, die zusätzlich zur Basistherapie bestehend aus Prednisolon (0,25-0,5 mg/kg/d), Dapson (1,0-1,5 mg/kg/d) und Clobetasolpropionat 0,05 % Salbe (läsional 2 x/d) mit Protein-A-IA (3 IAs an aufeinander folgenden Tagen) behandelt wurden. Die durchschnittliche Nachbeobachtungszeit betrug 33,6 Monate (1-84 Monate)., Ergebnisse: Bei der Mehrzahl der Patienten zeigte sich ein rascher und langandauernder Therapieeffekt. Nach einem Monat befanden sich acht Patienten (von 19 nachbeobachteten, 42 %) und zum Zeitpunkt des letzten Kontakts (nach 1-84 Monaten) 13 Patienten (68 %) in kompletter Remission. Die Anti-BP180-Autoantikörper wurden nicht nur initial (um 92 %) gesenkt, sondern lagen auch nach ein und drei Monaten im Mittel bei 26 % und 13 % des Wertes vor Therapiebeginn (p < 0,001). Bei Differenzierung in vortherapierte bzw. therapienaive Patienten zeigten sich in beiden Subgruppen signifikante Absenkungen der Anti-BP180NC16A-Antikörper-Spiegel zu allen Zeitpunkten. Unerwünschte Ereignisse traten bei dreizehn (65 %) der 20 Patienten auf, Drei der Ereignisse waren schwer und zwei davon wahrscheinlich oder möglicherweise in Zusammenhang mit der IA., Schlussfolgerungen: Die IA ist eine effektive adjuvante Therapieoption bei den in aller Regel älteren Patienten mit einem schweren und/oder therapierefraktären BP., (© 2018 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.)

Background: While depletion of circulating autoantibodies using immunoadsorption (IA) is an established therapeutic approach in patients with pemphigus vulgaris, IA has only sporadically been used in other autoimmune bullous disorders. Although bullous pemphigoid (BP) usually responds well to topical and systemic corticosteroids, rapid depletion of serum autoantibodies may be an effective adjuvant treatment option in patients with severe and/or refractory disease., Patients and Methods: Case series of 20 patients (13 women, 7 men; mean age 78.6 years; range 56-94 years) with severe or refractory BP. In addition to oral prednisolone (0.25-0.5 mg/kg/day), dapsone (1.0-1.5 mg/kg/day), and clobetasol propionate 0.05 % ointment (lesional application, twice daily), treatment consisted of protein A IA (three sessions on consecutive days). The mean follow-up period was 33.6 months (1-84 months)., Results: The majority of patients showed a rapid and sustained response. One month after treatment, eight patients (42 %; 19 patients were included in the follow-up) were in complete remission; at the last follow-up visit (after 1 to 84 months), that number was 13 (68 %). Not only was there an initial drop in anti-BP180 autoantibodies (by 92 %), the effect also continued after one and three months, with mean autoantibody levels at 26 % and 13 % of baseline, respectively (p < 0.001). Both previously treated and treatment-naive patients showed a significant reduction in anti-BP180NC16A antibody levels throughout the observation period. Adverse events occurred in 13 of the 20 patients (65 %). Three were severe of which two were likely or probably related to IA., Conclusion: Immunoadsorption is an effective adjuvant treatment option for (the usually elderly) patients with severe and/or refractory BP., (© 2018 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.)

Objectives: Various complaints of patients with fibromyalgia often resemble clinical features observed in patients with hypothalamic-pituitary diseases. The aim of this study was to evaluate whether patients with hypothalamic-pituitary diseases are at increased risk for fibromyalgia syndrome (FMS)., Methods: A questionnaire for evaluating fibromyalgia-associated symptoms was sent to 121 patients with hypothalamic-pituitary disorders (HPD) (60 women, 61 men; mean age, 55.4 years; range, 21-83 years) of the endocrine outpatient clinic. 115 patients (57 women, 58 men; mean age 56.9 years; range, 21 to 82 years) with cardiovascular diseases (CD) served as controls., Results: Fibromyalgia-associated symptoms regarding muscular complaints were significantly more frequent in the HPD group than in CD patients (53.7 % vs. 35.7%, p= 0.003). In particular, we found a significant higher prevalence of autonomic symptoms in the HPD group as compared to the CD group regarding several qualities (cold hands, p=0.039; flatulence, p=0.022; tiredness, p=0.017). In addition, swollen and painful finger joints were reported more often in the HPD group than in the CD group (p=0.002). Of note, no differences regarding any fibromyalgia-associated symptom were detected when patients with hypothalamic-pituitary hormone excess syndromes were compared to those with a pituitary pathology without hormonal excess. Similarly, prevalence of fibromyalgia-associated symptoms was not related to the treatment modality of pituitary disease; i.e. surgical vs. conservative or any hormonal replacement therapy., Conclusions: Our data suggest that patients with hypothalamic-pituitary disorders may be at increased risk of developing fibromyalgia-associated symptoms.

Objective: Low birth weight (LBW) is associated with obesity and a higher cardiovascular risk in adult life. Since autonomic dysfunction could be a pathophysiological factor for this association, we assessed the impact of LBW on cardiac autonomic activity and metabolic rate in young adulthood. We hypothesized that the autonomic tone could be coupled with the metabolic rate in subjects with LBW., Methods: Heart rate variability (HRV) from 24-hour Holter-electrocardiograms was measured in 15 healthy adults aged 20 to 30 years with LBW (<2500g at term) and 15 paired subjects with normal birth weight (NBW). The pairs were closely matched by gender, age, and body mass index. Resting energy expenditure was measured by indirect calorimetry and body composition by bioimpedance., Results: Global HRV parameters were significantly higher in the LBW group and a marked difference was observed in the long wave fluctuations of the frequency domain (very low frequency). These fluctuations were positively correlated with the resting energy expenditure per body weight in the LBW and negatively in the NBW group. Only in the LBW group HRV was closely related to body fat., Interpretation: This case-control study indicates that autonomous nervous function is not generally deteriorated in young adults with LBW and has a significant association with metabolic rate. Thus, it may be a determinant of the body weight regulation in this group. The higher cardiovascular risk in ageing individuals with LBW would therefore rather be a consequence of weight gain than of a primary autonomous nervous dysfunction.

Background and Purpose: Deterioration of cardiac autonomic nervous system in diabetics is associated with increased cardiac and arrhythmogenic mortality. Therefore, the present study engaged in the question how heart rate variability is acutely changed in diabetic ketoacidosis or hyperglycemic hyperosmolar syndrome. Moreover was evaluated how blood pressure, heart rate and incidence of arrhythmias can be explained by figures of heart rate variability., Patients and Methods: In a prospective observation of time course we investigated in 4 years consecutively 12 intensive care patients with DKA and 2 with HHS (10 male, 4 female, 19-62 years, initial plasma glucose 404-1192 mg/dl). All patients received a standardized treatment to international current guidelines. In addition to hemodynamic and clinical-chemical monitoring HRV analysis was performed continuously for at least 48 hours. Simultaneously, we determined supraventricular and ventricular arrhythmic episodes., Results: HRV was diminished over the whole spectrum in dependence on blood glucose concentration. Thus, sympathovagal balance (LF/HF ratio) was initially sympathetic predominated in blood glucose levels < 600 mg/dl (relatively prevailing LF power) and vagal predominated in blood glucose levels > 600 mg/dl (relatively prevailing HF power). In correlation analysis of HRV parameters with blood glucose rS-coefficients from -0.934 to -0.821 were achieved (p < 0.001). Further, the initial mean blood pressure correlated with the LF/HF ratio in HRV minimum (rS = 0.711, p = 0.004). The initial heart rate in relation to assumed intrinsic frequency correlated with minimal found Total Power (rS = -0.656, p = 0.011). In the period of whole 48 hours, more arrhythmic events occurred in consequence to initial glucose levels (rS = 0.693, p = 0.006). But the maximum of arrhythmic episodes was usually later ascertained than the minimum of HRV (p < 0.001). At the time of each arrhythmic maximum the sympathovagal balance (LF/HF) showed no uniform figures. Only similar in all cases was that the LF/HF ratio was found either > 4 or < 1., Conclusion: Clinical complications in high glucose levels must be seen in the context of a nearly complete blockade of sympathetic and parasympathetic activity. Basically to extreme autonomic restriction, sympathetic and vagal predominance can change rapidly into each other. This retarded vulnerable predisposition may declare the arrhythmic potential. An important progress in the monitoring of patients could be achieved by implementation of a continuous HRV measurement because hereby the actual risk potential can be ascertained timely and reliably.

Background: Alcohol intoxication is associated with deterioration of cardiac nervous function and increased mortality., Patients and Methods: Therefore, 14 patients (eight male, six female; 24-59 years) with pure ethanol intoxication and toxic levels of 210-520 mg/dl in the blood were prospectively investigated. For evaluation of changes in sympathetic and vagal modulation, an analysis of heart rate variability (HRV) was performed., Results: The results show that HRV in general is suppressed in dependence on blood alcohol levels. Most correlation was found in short-term variability of frequency domain (HF power) after logarithmic transformation (p < 0.001). Also the discrimination of moderate versus severe intoxication was greatest in HF power (p < 0.001). During recovery, all HRV parameters increased to significantly higher figures within 24 h (p < 0.001), but elevated LF/HF ratio as a sign of sympathetic predominance continued over the whole observation period (p < 0.001 to reference). Regarding the danger of possibly life-threatening arrhythmias, the LF/HF ratio showed significantly increased figures in the period of maximal development of supraventricular and ventricular arrhythmias (p = 0.001 to 24-h mean of LF/HF)., Conclusion: These results underline the longer-lasting hyperadrenergic state during "holiday heart syndrome". The still depressed cardiac autonomic nervous system after 24 h, especially in the vagal activity-describing part, might be basically relevant for later occurrence of cardiovascular complications, if additional trigger factors become present.