PubMed | University of Zürich, Kantonsspital Frauenfeld and Childrens Hospital
Type: | Journal: Journal of nephrology | Year: 2016
Inherited distal renal tubular acidosis (dRTA) is caused by impaired urinary acid excretion resulting in hyperchloremic metabolic acidosis. Although the glomerular filtration rate (GFR) is usually preserved, and hypertension and overt proteinuria are absent, it has to be considered that patients with dRTA also suffer from chronic kidney disease (CKD) with an increased risk for adverse pregnancy-related outcomes. Typical complications of dRTA include severe hypokalemia leading to cardiac arrhythmias and paralysis, nephrolithiasis and nephrocalcinosis. Several physiologic changes occur in normal pregnancy including alterations in acid-base and electrolyte homeostasis as well as in GFR. However, data on pregnancy in women with inherited dRTA are scarce. We report the course of pregnancy in three women with hereditary dRTA. Complications observed were severe metabolic acidosis, profound hypokalemia aggravated by hyperemesis gravidarum, recurrent urinary tract infection (UTI) and ureteric obstruction leading to renal failure. However, the outcome of all five pregnancies (1 pregnancy each for mothers n. 1 and 2; 3 pregnancies for mother n. 3) was excellent due to timely interventions. Our findings highlight the importance of close nephrologic monitoring of women with inherited dRTA during pregnancy. In addition to routine assessment of creatinine and proteinuria, caregivers should especially focus on acid-base status, plasma potassium and urinary tract infections. Patients should be screened for renal obstruction in the case of typical symptoms, UTI or renal failure. Furthermore, genetic identification of the underlying mutation may (a) support early nephrologic referral during pregnancy and a better management of the affected woman, and (b) help to avoid delayed diagnosis and reduce complications in affected newborns.
Zbinden A.,University of Zürich |
Quiblier C.,University of Zürich |
Hernandez D.,University of Geneva |
Herzog K.,Kantonsspital Frauenfeld |
And 5 more authors.
Journal of Clinical Microbiology | Year: 2014
Small-colony variants (SCVs) of bacteria are associated with recurrent and persistent infections. We describe for the first time SCVs of Streptococcus tigurinus in a patient with a prosthetic joint infection. S. tigurinus is a novel pathogen of the Streptococcus mitis group and causes invasive infections. We sought to characterize S. tigurinus SCVs using experimental methods and find possible genetic explanations for their phenotypes. The S. tigurinus SCVs were compared with the wild-type (WT) isolate using phenotypic methods, including growth under different conditions, autolysis, and visualization of the cell ultrastructure by use of transmission electron microscopy (TEM). Furthermore, comparative genome analyses were performed. The S. tigurinus SCVs displayed reduced growth compared to the WT and showed either a very stable or a fluctuating SCV phenotype. TEM analyses revealed major alterations in cell separation and morphological abnormalities, which were partially explained by impaired autolytic behavior. Intriguingly, the SCVs were more resistant to induced autolysis. Whole-genome sequencing revealed mutations in the genes involved in general cell metabolism, cell division, stringent response, and virulence. Clinically, the patient recovered after a 2-stage exchange of the prosthesis. Comparative whole-genome sequencing in clinical strains is a useful tool for identifying novel genetic signatures leading to the most persistent bacterial forms. The detection of viridans streptococcal SCVs is challenging in a clinical laboratory due to the small colony size. Thus, it is of major clinical importance for microbiologists and clinicians to be aware of viridans streptococcal SCVs, such as those of S. tigurinus, which lead to difficult-to-treat infections. Copyright © 2014, American Society for Microbiology. All Rights Reserved.
Winterhalder R.,Kantonsspital Luzern |
Hoesli P.,Etablissements Hospitaliers Nord Vaudois |
Delmore G.,Kantonsspital Frauenfeld |
Pederiva S.,Kantonsspital Baden |
And 3 more authors.
Oncology | Year: 2011
Objectives: While oral anticancer treatment has increased the convenience for patients with no risk of venous access complications compared to intravenous drug administration, a high level of compliance cannot always be assumed. The aim of the present report was to evaluate real-life drug adherence in a prospective cohort analysis of patients with gastrointestinal or breast cancer treated with capecitabine-based chemotherapy. Methods: Twenty-nine Swiss oncologists recruited patients receiving capecitabine, either as monotherapy or in combination with other chemotherapeutic agents, in a prospective fashion. Patients recorded both their capecitabine intake and any adverse effects each day in patient diaries. Results: A total of 177 patients were included, 143 (81%) with gastrointestinal tumours and 34 (19%) with breast cancer. Overall, 161 patients (91%) were considered as fully compliant, while 16 patients (9%) reported some kind of compliance error. Reasons for non-compliance included forgetting to take treatment (n = 9), side effects (n = 4) and misunderstanding instructions (n = 3). Self-reported compliance was not influenced by age or Eastern Cooperative Oncology Group performance status, but there was a trend towards better compliance with capecitabine therapy if fewer adverse effects occurred (p = 0.07, simple logistic regression). Conclusions: Self-reported compliance with capecitabine-based therapy in clinical practice is high and seems to be adversely affected by side effects. © 2011 S. Karger AG, Basel.
Bekou V.,Vein Clinic Bellevue |
Zollikofer C.,Kantonsspital Frauenfeld |
Nieuwkamp N.,Kantonsspital Frauenfeld |
von Weymarn A.,Kantonsspital Frauenfeld |
And 2 more authors.
Phlebology | Year: 2014
Objectives: The nutcracker syndrome (NS) may lead to insufficient perirenal collaterals as well as incompetence of the left ovarian vein with consecutive ovarian vein insufficiency. Methods: A female patient with NS and severe genital varicosis was treated with dilation of the renal vein and coiling of a left perirenal collateral vein feeding an insufficient left ovarian vein (LOV) with pelvic vein varicosity. Results: In re-evaluation 18 month later with left renal vein (LRV) and LOV phlebography, a widely patent LRV was found. The embolized LRV to LOV collateral was occluded. However, left hypogastric phlebography showed incompetent branches of the left hypogastric vein feeding the genital varicose veins. These were successfully embolized with coils and the genital varicosity decreased on follow-up. Conclusions: In our patient a combined therapeutic approach with balloon dilation of the NS and embolization of the genital varicose veins by left hypogastric vein coil was performed.
Zettl R.,Kantonsspital Frauenfeld |
Eschbach D.,Justus Liebig University |
Ruchholtz S.,Justus Liebig University
International Orthopaedics | Year: 2015
Purpose: Periprosthetic acetabular fractures are rare and in the current literature largely underreported. The management is reported to be difficult. Treatment varies from non-operative to open reduction and internal fixation up to revision of the acetabular components. Methods: A prospective consecutive case series in acetabular fractures was performed in a level 1 trauma centre. All patients with pre-existing total hip replacement were followed up for one year. Perioperative data, complications, radiological results, functional outcome and quality of life were measured. Results: Eight (15 %) of 53 patients who were included in the study underwent total hip arthroplasty before and had stable implants at time of fracture. Mean age of the patients was 83 years. All of them were female. Mean operative time was 85 minutes. There were no soft tissue complications like infection or nerve damage in the post-operative course. No revision was needed. Two patients died in between the follow up. The Harris hip score was a mean of 77, with quality of life comparable to persons in the same age. Conclusions: Minimally invasive reconstruction of the anterior column is a viable method to conserve stable acetabular components in this type of fracture. Short operation time and limited incisions are the most conclusive advantages. © 2015, SICOT aisbl.
Monitoring for potential residual disease activity by serum insulin-like growth factor 1 and soluble Klotho in patients with acromegaly after pituitary surgery: Is there an impact of the genomic deletion of exon 3 in the growth hormone receptor (d3-GHR) gene on "safe" GH cut-off values?
Kohler S.,University of Zürich |
Tschopp O.,University of Zürich |
Sze L.,University of Zürich |
Neidert M.,University of Zürich |
And 4 more authors.
General and Comparative Endocrinology | Year: 2013
Background: Acromegaly is an illness usually defined by excessively high growth hormone (GH) and insulin like growth factor 1 (IGF-1) levels, the latter mainly reflecting GH action on the liver. IGF-1, also known as somatomedin C, mediates several actions of GH. The diagnosis and management of acromegaly is relatively straight forward, but long-term follow-up of patients can be difficult, as elevated IGF-1 levels can occur in the presence of apparently normalised GH levels and late recurrence of acromegaly may arise despite previous suppression on oral glucose tolerance testing. Data suggest this applies especially to patients in whom the GH receptor lacks exon 3. In such patients, GH may not always be a useful marker of disease, and traditional GH cut-offs may be misleading. Recent data suggest that soluble Klotho (sKlotho), besides and in addition to IGF-1, may help monitor the activity of GH-producing adenomas (presumably reflecting GH action on the kidneys) and may be a useful supplementary tool. Methods: GHR genotyping was performed in 112 patients with acromegaly. IGF-1 and sKlotho levels were measured in the sera of patients before and after transsphenoidal surgery, with emphasis on patients judged inconclusively cured by surgery or with small residual tumour masses shortly after surgery. Patients were assessed for recurrence of acromegaly with GH levels (random or nadir during an oGTT). Results: Of the 48 patients who underwent surgery between 2000 and 2009 and who had well-documented longer term follow-up at our institution, 29 had no biochemical evidence of residual disease activity after transsphenoidal surgery (marked reduction in IGF-1 and sKlotho levels, GH suppressible to <1. ng/ml) and were classified as in remission. 2 of these patients developed recurrent symptoms of acromegaly during follow-up with increasing levels of IGF-1 and sKlotho, and both patients were carriers of the d3-GHR genotype. Conclusions: Acromegalic patients with the d3-GHR polymorphism might be - for a given low postsurgical GH level - at higher risk for recurrence and may require a lower GH nadir during oGTT to be classified as in remission. Soluble Klotho could be useful in the follow-up of acromegalic patients. The question arises whether sKlotho not only reflects the activity of GH-secreting pituitary adenomas but whether Klotho (ectodomain clipping?) could also mediate selected actions of GH. © 2013 The Authors.
Sze L.,University of Zürich |
Bernays R.L.,University of Zürich |
Zwimpfer C.,University of Zürich |
Wiesli P.,Kantonsspital Frauenfeld |
And 2 more authors.
Journal of Internal Medicine | Year: 2012
Objectives. Klotho-deficient mice develop a syndrome resembling accelerated ageing, and genetic variants of Klotho have been associated with human ageing. In humans, serum levels of soluble Klotho decrease with age and with chronic renal failure. The aim of our study was to examine the relationship between excess growth hormone (GH) and serum levels of Klotho in patients with acromegaly, a disease usually caused by a pituitary adenoma, which is associated with high phosphate levels and reduced life expectancy. Patients and design. We determined the levels of soluble Klotho, GH and insulin-like growth factor 1 (IGF-1) in serum samples from 24 consecutive patients with acromegaly (nine women/15 men, age 28-76years) before and after transsphenoidal surgery. Results. Soluble Klotho levels were excessively high at baseline (mean±SEM, 4.2±0.7ngmL -1) and correlated with GH (r=0.64), IGF-1 (r=0.57) and tumour size (r=0.5). In multiple regression analysis, soluble Klotho was associated with GH after correction for age, gender and levels of creatinine and phosphate (P=0.029). After surgery, GH and IGF-1 levels decreased in all patients (from 26.3±5.2 to 2.6±0.6μgL -1, P <0.0001, and from 588±35 to 193±12μgL -1, P<0.001, 0.0001, respectively). Creatinine increased from 71±3 to 80±3μmolL -1 (P<0.001), and phosphate decreased from 1.37±0.04 to 1.06± 0.02mmolL -1 (P<0.001). The markedly increased preoperative levels of soluble Klotho returned towards normal after surgery (0.7±0.1ngmL -1, P<0.0001). Conclusions. This is the first study to show dramatically increased soluble Klotho levels in an acquired disease in humans. Reversal following tumour removal suggests a causal relation between the GH-producing adenoma and high serum Klotho concentration in acromegaly. © 2012 The Association for the Publication of the Journal of Internal Medicine.
Faulenbach M.,University of Zürich |
Uthoff H.,Kantonsspital Frauenfeld |
Uthoff H.,University of Basel |
Schwegler K.,University of Zürich |
And 3 more authors.
Diabetic Medicine | Year: 2012
Aim To investigate the effect of acute psychological stress on glucose concentrations in patients with Type2 diabetes, in the fasting state as well as in the postprandial state. Methods Thirty patients (12 female) with Type2 diabetes were included. Mean±sd age was 60±12years, BMI 28.8±4.2kg/m 2, diabetes duration 8.9±6.7years and HbA 1c 51±9mmol/mol (6.8±0.8%). Using a non-randomized approach, all participants were exposed to moderate psychological stress by means of the Trier Social Stress Test: 10 participants in the fasting state and 20 participants 75min after intake of a standard meal. Blood pressure, heart rate and salivary cortisol were monitored on the control day and the stress-test day. Glucose concentrations were assessed using a continuous glucose monitoring system. Results On the stress-test day, blood pressure rose from 117/73±13/12 to 155/92±22/14mmHg, heart rate from 77±11 to 91±25bmin -1 and salivary cortisol concentrations from 8.5±3.7 to 26.4±12.1nmol/l (P<0.001); these measurements remained unchanged on the control day. On the stress-test day, when the Trier Social Stress Test was applied 75min after the intake of a standard meal, the glucose concentrations were significantly higher compared with the control day (mean difference 1.5mmol/l, 95%CI 0.5-2.4, P=0.003). In the fasting state, glucose concentrations slightly decreased during the control day but remained stable on the stress-test day (mean difference compared with the control day 0.7mmol/l, 95%CI -0.7 to 2.0, P=0.31). Conclusions When stress is experienced in the postprandial period, acute psychological stress significantly increases glucose concentrations in patients with Type2 diabetes. © 2011 The Authors. Diabetic Medicine © 2011 Diabetes UK.
Gamper M.,Institute For Klinische Biomedizinische Forschung Thurgau |
Viereck V.,Kantonsspital Frauenfeld |
Eberhard J.,Kantonsspital Frauenfeld |
Binder J.,Kantonsspital Frauenfeld |
And 3 more authors.
International Urogynecology Journal and Pelvic Floor Dysfunction | Year: 2013
Introduction and hypothesis Bladder pain syndrome/interstitial cystitis (BPS/IC) is identified based on subjective symptoms which lead to heterogeneous patient populations. Previous studies using gene expression arrays for BPS/IC with Hunner's lesions [European Society for the Study of Interstitial Cystitis (ESSIC) type 3C], a subtype of the condition discernible by cystoscopy, have revealed characteristic immune responses and urothelial abnormalities. This current study aimed to further characterize this subtype using a gene expression panel. We hypothesized that B-cell activation with high levels of urinary antibody concentration would be found. Methods Cold-cup bladder biopsies, catheterized urine and blood were collected from 15 BPS/IC ESSIC type 3C patients, 11 non-inflammatory overactive bladder (OAB) patients and eight healthy controls. Gene expression in biopsies was quantified by real-time quantitative polymerase chain reaction (RT-qPCR), immunohistochemistry was performed on bladder tissue and urinary immunoglobulins G and A were quantified by enzyme-linked immunosorbent assay. Statistical analyses included the Kruskal-Wallis test for non-parametric data and post hoc tests identified differences between groups. Results High expression of T- and B-cell markers (CTLA4, CD20, CD79A, IGH), low expression of urothelial markers (KRT20, UPK1B, UPK3A), focal lymphoid aggregates in the submucosa and high immunoglobulin concentration in urine were found exclusively in BPS/IC ESSIC type 3C patients. Results for OAB were in intermediate ranges between the other two groups and UPK1B even reached significantly lower expression when compared to healthy controls. Conclusions BPS/IC ESSIC type 3C is characterized by a local adaptive immune response with elevated urinary antibody concentrations. Quantification of urinary immunoglobulin levels could be used for a non-invasive diagnosis of BPS/IC ESSIC type 3C. © The Author(s) 2013.
PubMed | University of Zürich and Kantonsspital Frauenfeld
Type: Journal Article | Journal: Journal of wrist surgery | Year: 2016
PurposePatients with symptomatic instability of the distal radioulnar joint (DRUJ) after traumatic complete disruption of the triangular fibrocartilage complex (TFC) are best treated by anatomic reconstruction of the TFC. Postoperative clinical results from the literature are known but the improvement of DRUJ instability remains still challenging to quantify. We recently published a reliable and validated method to measure the instability of the DRUJ. This sonographic method was used to quantify the pre- and postoperative instability in correlation with clinical outcome in patients with complete TFC disruption. Methods11 patients with complete disruption of the TFC resulting in symptomatic instability of the DRUJ underwent open reconstruction of the TFC. The instability was measured with sonography preoperatively and one year postoperatively including the Patient-Rated Wrist Evaluation score (PRWE Score). ResultsBy subjective measurement, 9 patients showed comparable stability to the contralateral side. By objective measurements, DRUJ stability was completely restored in 6 patients. Seven patients had a very good and good clinical outcome. The dorsovolar shift (preoperative mean 5.2mm, min 2.4, max 7.1; postoperative 3.6mm, min 1.2, max 6.2) was significantly decreased (p<0.05) and was postoperatively not different to the contralateral healthy side (p>0.1). PRWE score in the 1 year follow up was 13.8. Three patients remained with significant pain, sonographically two of them were still more lax and one tighter compared to the contralateral side. ConclusionsThe sonographic measurement technique allows evaluation of the stability pre- and postoperatively and does not always correlate with the qualitative clinical assessment. The described operation technique is effective for treatment of irreplaceable TFC ruptures and significantly improves the DRUJ stability and wrist function one year after surgery, which could be quantified objectively by ultrasound.