[Depressive and anxiety symptoms in hereditary connective tissue disorders : case description and systematic literature review]. / Depressieve en angstklachten bij erfelijke bindweefselaandoeningen: casus en systematisch literatuuroverzicht.

Hereditary connective tissue disorders are a broad group of congenital disorders that are characterized by a pathological weakness of the connective tissue as a result of an incorrect genesis, leading to multisystem complaints. We describe a 14-year-old patient with the hereditary connective tissue disorder Loeys-Dietz syndrome who was admitted to a child psychiatric crisis unit because of depressive and anxiety symptoms. A systematic literature search was carried out to analyze the prevalence of depressive and anxiety symptoms in individuals with hereditary connective tissue disorders Loeys-Dietz syndrome, Ehlers-Danlos syndrome and Marfan syndrome, to identify a possible association between these disorders and explanations for this. We conclude that there is an increased incidence of depression and anxiety symptoms in which pain, fatigue, social support and functioning, quality of life and functional limitations seem to play a role. There is a need for further research to determine exactly which factors contribute and how these can be targeted in prevention and treatment.

Diagnostic value of the electrocardiogram in the assessment of prior myocardial infarction.

BACKGROUND: The best available imaging technique for the detection of prior myocardial infarction (MI) is cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE). Although the electrocardiogram (ECG) still plays a major role in the diagnosis of prior MI, the diagnostic value of the ECG remains uncertain. This study evaluates the diagnostic value of the ECG in the assessment of prior MI. METHODS: In this retrospective study, data from electronic patient files were collected of 1033 patients who had undergone CMR with LGE between January 2014 and December 2017. After the exclusion of 59 patients, the data of 974 patients were analysed. Twelve-lead ECGs were blinded and evaluated for signs of prior MI by two cardiologists separately. Disagreement in interpretation was resolved by the judgement of a third cardiologist. Outcomes of CMR with LGE were used as the gold standard. RESULTS: The sensitivity of the ECG in the detection of MI was 38.0% with a 95% confidence interval (CI) of 31.6-44.8%. The specificity was 86.9% (95% CI 84.4-89.1%). The positive and negative predictive value were 43.6% (95% CI 36.4-50.9%) and 84.0% (95% CI 81.4-86.5%) respectively. In 170 ECGs (17.5%), the two cardiologists disagreed on the presence or absence of MI. Inter-rater variability was moderate (κ 0.51, 95% CI 0.45-0.58, p < 0.001). CONCLUSION: The ECG has a low diagnostic value in the detection of prior MI. However, if the ECG shows no signs of prior MI, the absence of MI is likely. This study confirms that a history of MI should not be based solely on an ECG.

Neurostimulation in neurogenic patients.

PURPOSE OF REVIEW: To provide an overview of available electrical stimulation devices in neurogenic patients with lower urinary tract disease. RECENT FINDINGS: It is advocated to do more studies in neurogenic patients as results seem promising and useful but most studies did not include neurogenic patients or neurogenic patients were not analyzed or reported separately. Most studies included a small heterogenous neurogenic group with multiple pathophysiologic origin focusing on effect of a treatment instead of results of a treatment in a specific neurogenic group. Neuromodulation or stimulation has the advantage that it acts on different organs, like bladder and bowel, so can treat neurogenic patients, who mostly suffer from multiple organ failure. SUMMARY: Brindley procedure, sacral neuromodulation (SNM) and posterior tibial nerve stimulation (PTNS) are available for a while already. The Brindley procedure (including sacral anterior root stimulation in combination with a rhizotomy of posterior sacral roots) is developed for selected spinal cord injury patient with a complete spinal injury, and has shown results for many years in neurogenic patients. An alternative to the rhizotomy is not established yet. SNM and PTNS are other modalities that are used in nonneurogenic patients, but are not yet indicated and much studied in neurogenic patients.

Survival of the artificial urinary sphincter in a changing patient profile.

PURPOSE: To examine the functional survival of the artificial urinary sphincter (AUS) AMS800 in a changing patient population. Because of increasing experience and dexterity of the operating team, we hypothesize that patients with known risk factors nowadays have a better survival of their prosthesis. However, due to a change to a more complex case mix, overall results appear to be worse. MATERIALS AND METHODS: All men who underwent implantation of an AUS between 2001 and 2016 because of urethral sphincter deficiency were retrospectively analyzed. Patients were divided in groups based on date of surgery and number of patients: 2001-2009 (G1), 2010-2013 (G2), 2014-2016 (G3). Baseline characteristics and additional therapies prior to implantation were analyzed in all groups. Risk factors for failure only in G1 and G2. Revision or explantation of the AUS was used as endpoint. Kaplan-Meier analysis was used to calculate survival of the device. RESULTS: A total of 129 patients (mean age 72 ± 9 years) underwent 129 primary implants, and 11 secondary implants. Median follow-up was 5.74 years in G1, 3.26 years G2 and 1.54 years G3. Approximately 25% of the patients in G1 had received adjuvant therapy for prostate cancer and 14% underwent previous surgery for incontinence. In G2, 51 and 55% underwent adjuvant therapy for prostate cancer and previous surgery for incontinence, respectively, G3 was comparable. The overall 50% survival improved in patients with radiotherapy and previous incontinence surgery in G2 as compared to G1. CONCLUSIONS: Despite the more complex patient population, the survival of the AUS did not decrease. In some patient categories, the AUS functional survival is even still improving over the past few years.

Negative predictive value of SPECT for the occurrence of MACE in a medium-sized clinic in the Netherlands.

BACKGROUND: Single-photon emission computed tomography (SPECT) is an important prognostic tool in evaluating coronary artery disease (CAD), with a high negative predictive value (NPV) for the occurrence of major adverse cardiac events (MACE). The prognostic value of SPECT is disputed in women, patients with atrial fibrillation (AF), diabetes, left bundle branch block (LBBB) and renal impairment. METHODS: Seven hundred sixty-two patients without prior history of CAD who had SPECT without perfusion deficits were followed for 2 years for MACE. Predictive variables for the occurrence of MACE were reviewed by Cox proportional hazard regression, considering clinical information, resting-ECG data and SPECT data. RESULTS: The NPV of SPECT for the occurrence of MACE within 2 years was 95.8 %. Multivariate Cox regression revealed male gender as the only significant predictor for the occurrence of MACE, besides a positive stress ECG at SPECT and a low LVEF. AF, LBBB, renal impairment and diabetes had no significant effect on the prognosis after normal SPECT. CONCLUSION: SPECT with normal perfusion images has great NPV in a medium-sized clinic in the Netherlands, even in patients with LBBB, AF, diabetes and renal impairment. MACE-free survival, however, was negatively influenced by male gender; we therefore propose more caution in men.

[Treatment practice in patients with isolated blunt splenic injuries. A survey of Swiss traumatologists]. / Die Behandlungspraxis bei Patienten mit isolierter stumpfer Milzverletzung. Eine Befragung der Schweizer Traumatologen.

BACKGROUND: The non-operative management (NOM) of blunt splenic injuries has gained widespread acceptance. However, there are still many controversies regarding follow-up of these patients. The purpose of this study was to survey active members of the Swiss Society of General and Trauma Surgery (SGAUC) to determine their practices regarding the NOM of isolated splenic injuries. MATERIALS AND METHODS: A survey of active SGAUC members with a written questionnaire was carried out. The questionnaire was designed to elicit information about personal and facility demographics, diagnostic practices, in-hospital management, preferred follow-up imaging and return to activity. RESULTS: Out of 165 SGAUC members 52 (31.5%) completed the survey and 62.8% of all main trauma facilities in Switzerland were covered by the sample. Of the respondents 14 (26.9%) have a protocol in place for treating patients with splenic injuries. For initial imaging in hemodynamically stable patients 82.7% of respondents preferred ultrasonography (US). In cases of suspected splenic injury 19.2% of respondents would abstain from further imaging. In cases of contrast extravasation from the spleen half of the respondents would take no specific action. For low-grade injuries 86.5% chose to admit patients for an average of 1.6 days (range 0-4 days) with a continuously monitored bed. No differences in post-discharge activity restrictions between moderate and high-grade splenic injuries were found. CONCLUSION: The present survey showed considerable practice variation in several important aspects of the NOM of splenic injuries. Not performing further CT scans in patients with suspected splenic injuries and not intervening in cases of a contrast extravasation were the most important discrepancies to the current literature. Standardization of the NOM of splenic injuries may be of great benefit for both surgeons and patients.

Minimal invasive electrode implantation for conditional stimulation of the dorsal genital nerve in neurogenic detrusor overactivity.

STUDY DESIGN: Experimental. OBJECTIVES: Electrical stimulation of the dorsal genital nerves (DGN) suppresses involuntary detrusor contractions (IDCs) in patients with neurogenic detrusor overactivity (DO). The feasibility of minimal invasive electrode implantation near the DGN and the effectiveness of conditional stimulation to suppress IDCs at different amplitudes in spinal cord injury (SCI) patients with DO were studied. SETTING: Radboud University Nijmegen MC, The Netherlands. METHODS: In eight healthy volunteers, a needle electrode was inserted from both a medial and lateral-to-midline site at the level of the pubic bone. Electrode insertion was guided by the genito-anal reflex (GAR) evoked by electrical stimulation and by sensation to this stimulation. In eight SCI patients with DO, the bladder was repeatedly filled and emptied partially in between. Conditional stimulation using a needle electrode was applied when an IDC was observed at urodynamics. Different amplitudes were used during each filling. Control cystometry was carried out before electrode insertion and after stimulation. RESULTS: The lateral implant approach was preferred, as it was easier to manoeuvre the needle along the pubic bone and fixate the needle. In SCI patients, the electrode was positioned successfully, and IDCs were suppressed (range 1-6 IDC suppressions) with conditional stimulation at maximum tolerable amplitude, except for one patient. Stimulation was less effective at lower amplitudes. Stimulation lowered the intensity of bladder sensations concomitant with IDC. CONCLUSION: The lateral-to-midline implant approach, in combination with GAR and sensation to stimulation, is feasible for electrode implantation near the DGN in SCI patients. Conditional stimulation effectively suppresses IDCs.

Limited value of bladder sensation as a trigger for conditional neurostimulation in spinal cord injury patients.

AIMS: Conditional stimulation of dorsal genital nerves suppresses undesired detrusor contractions (UDC) and consequently increases bladder capacity and prevents incontinence. No clinically applicable sensor exists for reliable bladder activity monitoring as a trigger for conditional stimulation. Primary objective of this study was to determine whether bladder sensation concomitant with UDC may be used for spinal cord injury (SCI) patients to trigger neurostimulation in daily life. METHODS: Nineteen male and 7 female SCI patients suspected of detrusor overactivity (DO) underwent conventional and 6-hr ambulatory urodynamics. Patients were instructed to do normal daily activities and to activate event buttons of the ambulatory recorder to mark events: physical activity, bladder sensation, micturition or intermittent catheterization, and urinary incontinence. Detection rate was defined as the number of recorded bladder sensation divided by the total number of recorded UDC during ambulatory urodynamics. RESULTS: Bladder sensation was reported by 73% of patients in daily life. Only 41% of patients had analyzable bladder sensation concomitant with UDC during ambulatory urodynamics. For ambulatory and conventional urodynamics, mean detection rates were 23% and 72%, respectively, with mean recording delays of 57 and 16 sec after UDC onset, respectively. CONCLUSIONS: Bladder sensation only occurs in a small group of SCI patients combined with a rather low detection rate and long reaction time. Therefore, bladder sensation as a trigger for conditional stimulation does not seem to be suitable for SCI patients with DO. Reliable techniques for chronic bladder activity monitoring are a prerequisite for successful clinical application of conditional stimulation.

No primary role of ambulatory urodynamics for the management of spinal cord injury patients compared to conventional urodynamics.

AIMS: Adequate urodynamic assessment of bladder behavior is essential in spinal cord injury (SCI) patients. Ambulatory urodynamics are more sensitive to detect detrusor overactivity (DO) than conventional urodynamics. The primary objective of this study was to determine the value of ambulatory urodynamics for the diagnosis of DO in SCI patients compared to conventional urodynamics. METHODS: Twenty-seven SCI patients who were suspected of DO underwent both conventional and ambulatory urodynamics at one day. A single involuntary detrusor contraction (IDC) was defined as a detrusor pressure rise of at least 10 cmH(2)O. DO according to the ICS definition was used in addition to minimize the influence of catheter artifacts. Outcome of urodynamics was used for decisions on treatment. RESULTS: Ambulatory urodynamics were more sensitive to diagnose IDC and DO. Conventional urodynamics had a sensitivity of 82% and specificity of 75% for DO diagnosis compared to ambulatory urodynamics. Mean maximum detrusor pressures did not differ significantly between both urodynamics. When the maximum detrusor pressure at conventional urodynamics did not exceed 40 cmH(2)O, 83% (10/12) of patients had a mean maximum detrusor pressure under 40 cmH(2)O at ambulatory urodynamics. Although the inter-individual DO diagnostic agreement was lower for ambulatory than conventional urodynamics (58%, K = 0.201 vs. 77%, K = 0552), the treatment agreement was higher for ambulatory urodynamics (58% vs. 42%). CONCLUSIONS: Ambulatory urodynamics do not seem necessary for diagnosis and risk assessment in SCI patients suspected for DO when conventional urodynamics are done properly. The exact role of urodynamics in treatment decision remains to be determined.

ProACT for stress urinary incontinence after radical prostatectomy.

INTRODUCTION: Stress urinary incontinence is a bothersome complication of radical prostatectomy. Surgical treatment consists of the artificial urinary sphincter (AUS), the male sling and bulk injections. This study presents the results of the first series of implantations of ProACT in the Netherlands. MATERIALS AND METHODS: A non-validated questionnaire was sent to 29 male patients implanted with ProACT to determine Stamey score, pad count and questions about quality of life and satisfaction. Complications, revisions and explantations were registered. RESULTS: Mean follow-up was 41 months. Based on Stamey score four patients are continent at the end and nine patients according to the pad count. The average pad count decreased significantly. Remarkable was the high rate of dislocations and revisions and patients' satisfaction. CONCLUSIONS: ProACT is a less invasive treatment compared to the AUS. However, the procedure is associated with a substantial revision and explantation rate. ProACT can be part of a so-called step-up approach before opting for a more invasive treatment.

Accidental intoxication with Veratrum album.

A 49-year-old man consumed two glasses (approximately 2 x 20 mL) of a beverage containing yellow gentian (Gentiana lutea). Shortly after ingestion, he developed nausea, vomiting, and oral paraesthesia. On admission to the hospital he suffered from severe bradycardia (35 beats/min) and hypotension (50/30 mm Hg), and he was treated with activated charcoal, antiemetics (metoclopramide, ondansetron), atropine, and intravenous electrolytic solution. The initial suspicion of Veratrum poisoning could be confirmed by identifying protoveratrines A (ProA) and protoveratrine B (ProB) in a sample from the beverage as well as in the patients serum by liquid chromatography-electrospray ionization-tandem mass spectrometry (LC-ESI-MS-MS). The yellow-colored beverage contained 25% ethanol (by headspace gas chromatography), 20.4 mg/L ProA, and 13.7 mg/L ProB. The serum concentration of ProA was 1162 ng/L and ProB was 402 ng/L. Veratridine, cevadine, and jervine were not detected, neither in the beverage nor in the serum sample. The lower limits of quantitation for all compounds is 10 microg/L (S/N > 10, beverage) and 100 ng/L (S/N > 10, serum). After treatment, the patient completely recovered from the symptoms within 24 h and was discharged from the hospital. The analytical method described was developed for the simultaneous identification and quantitation of five Veratrum alkaloids. The method is based on a liquid-liquid extraction followed by LC-MS-MS analysis. The time needed for analysis was 6 min.

Urinary gonadotropin measurements in neonates: a valuable non-invasive method.

BACKGROUND: To measure low neonatal gonadotropin levels, a sensitive non-invasive method is optimal. The aim of the current study was to validate the Architect i2000SR, an automated immunoassay analyser for the measurement of gonadotropins in unextracted neonatal urine samples against serum gonadotropin levels as a gold standard. METHODS: Blood and urine were sampled from 30 approximately six-week-old male and female neonates undergoing elective paediatric surgery. Follicle stimulating hormone (FSH) and luteinizing hormone (LH) were measured and the urine results were corrected for creatinine. RESULTS: The agreement between neonatal serum and urinary FSH was 0.904 (3-5 h between samples) and 0.704 (18-20 h). For LH, the correlation coefficients were 0.785 and 0.507, respectively. CONCLUSION: We conclude that gonadotropins can be reliably measured using the Architect on randomly voided, non-extracted urine samples collected from neonates by an adhesive device. Urinary gonadotropin levels are a proper reflection of the serum levels.

[The metabolic syndrome: a cluster of vascular risk factors]. / Het metabool syndroom: een cluster van vasculaire risicofactoren.

The metabolic syndrome is a cluster of several vascular risk factors (impaired glucose metabolism, dyslipidaemia, hypertension and central adiposity). The prevalence of the metabolic syndrome is high, varying between 10 and 40% depending on age and sex. This prevalence will increase in the years to come due to the increased prevalence of overweight/obesity. To identify the metabolic syndrome, there is a readily applicable definition for daily clinical practice, i.e. the presence of three or more of the following characteristics: hyperglycaemia, hypertension, low plasma HDL cholesterol level, high plasma triglyceride level and central adiposity. The underlying pathophysiology is not fully clarified, but insulin resistance plays an important role in this syndrome. The metabolic syndrome is associated with increased cardiovascular morbidity and mortality and an increased risk for the development of diabetes mellitus type 2. In subjects with one or two components of the metabolic syndrome and in patients with manifest vascular disease, it seems advisable to be alert to the presence of the other components in order to either diagnose or exclude the metabolic syndrome. Although clinical evidence is lacking, from a pathophysiological point of view it seems reasonable to focus the treatment on reducing insulin resistance, which can be achieved by weight reduction and an increase in physical activity. Treatment of the individual risk factors may also be considered, depending on the degree of vascular risk.

Transient involvement of endothelin in hypertrophic remodeling of small arteries.

OBJECTIVE: This study was designed to evaluate the capacity of norepinephrine (NE) to induce hypertrophic remodeling of small arteries in rats, and to determine the involvement of endothelin (ET) to initiate and maintain it. DESIGN AND RESULTS: Treatment with NE (2.5 microg/kg per min) for 14 or 28 days produced a similar inward hypertrophic remodeling, characterized by a smaller lumen, but increased media thickness and cross-sectional area. Arterial stiffness was reduced. Histological evaluation confirmed the hypertrophic nature of remodeling. Concomitant administration of LU135252 (ET-receptor antagonist) for the first 14 days of NE administration prevented the development of hypertrophy, without altering arterial mechanics. Treatment with the same antagonist from day 14 to day 28 of NE or angiotensin II (Ang II) treatment failed to regress established vascular hypertrophy. In contrast, normalization of arterial structure was observed with prazosin, an alpha-adrenergic blocker. Endothelin content in small mesenteric arteries showed a transient elevation following chronic NE administration. CONCLUSIONS: Increased circulating NE levels are associated with hypertrophic remodeling of small arteries, in which ET plays an initiating role. However, the maintenance of vascular hypertrophy is ET-independent, either in the presence of augmented circulating levels of NE or Ang II. Thus, early rather than late treatment with ET-receptor antagonists may be a preferable approach to limit small artery-mediated end-organ damage in cardiovascular diseases.

Hemoperfusion in acute camphor poisoning.

After ingestion of an unknown dose of a 10% camphor spirit (maximum dose 200 ml), a 54-year-old female was found in coma, having developed grand-mal-like seizures and respiratory failure. For detoxification, gastric lavage and hemoperfusion with amberlite XAD4 were performed. During hemoperfusion, seizures ceased, but no marked influence on the clinical course of the intoxication--especially on grade of coma--was observed. For further evaluation of the efficacy of treatment, camphor plasma elimination kinetics during hemoperfusion were compared to plasma elimination kinetics determined in a volunteer after an oral dose of 200 mg of camphor. Although the plasma compartment was almost completely (89%-95%) cleared of camphor by hemoperfusion, only a total of 35 mg of camphor was removed due to its high apparent volume of distribution (estimated 2-4 l/kg). The plasma elimination half-life was not affected by hemoperfusion, which may be explained by the saturation of metabolizing enzymes, still incomplete absorption, or metabolic interference of isopropanol. In the case presented here, neither the clinical course of the intoxication nor plasma level data gave evidence for a pronounced beneficial effect of hemoperfusion.

Mass concentration measurements of creatine kinase BB isoenzyme as an index of brain tissue damage.

A new two-site immunoenzymometric method using monoclonal antibodies was developed for measuring CK-BB mass concentrations in cerebrospinal fluid (CSF). Within- and between-assay coefficient of variation values for the method varied between 6 and 9%. Assay results are not affected by presence of sulfate and sialic acid groups on the enzyme. In comparison to catalytic activity measurements, a steady decline in the enzyme's specific activity was observed after acute head trauma. Repetitive measurements of CK-BB mass concentration in cerebrospinal fluid during the first 24 h after trauma enabled the estimation of brain lesion size. Clinical outcome of acute head trauma patients evaluated by Glasglow Outcome Scale, correlated well with cumulative CK-BB release after trauma. Also in neonates, CK-BB determinations in CSF correlated well with clinical findings.

Transesophageal echocardiography: a simple method for monitoring the patency of ventriculoatrial shunts. Technical note.

A new method for evaluating the patency of a ventriculoatrial shunt is described, and early experience with it is reported. Transesophageal echocardiography can demonstrate a cerebrospinal fluid leak in the right atrium through the atrial tip of a shunting device. This capability was an incidental discovery, and since then the accuracy of the technique in evaluating the patency of a ventriculoatrial shunt has been prospectively studied in 20 observations of 16 patients. The method proved to be accurate in 90% to 100% of cases. It is concluded that transesophageal echocardiography offers a rapid and accurate assessment of ventriculoatrial shunt function, is well tolerated, and is easy to perform.

Spinal cord stimulation in chronic pain therapy.

Spinal cord stimulation was undertaken in 45 patients referred to the University Hospital in Ghent. Failed back surgery was the major indication for implantation. Raynaud's phenomenon, causalgia, polyneuropathy, phantom limb pain, and diverse causes were the other indications. Before neurosurgical implantation of the system, a percutaneous epidural trial procedure was performed. The efficacy of the implanted stimulation system was estimated by considering the use of medication and the patients' personal appreciation of the obtained pain relief. Thirty-five patients experienced very good pain relief. Only two patients needed further narcotic analgesics. Eight patients stopped using the stimulation system. To ensure good results, strict selection criteria and many surgical reinterventions seemed to be necessary. Although spinal cord stimulation is a nonablative technique, many complications may occur.

Extraction and GLC analysis of pyrithyldione in highly putrified human postmortem samples.

Pyrithyldione was isolated from highly putrified human brain and small intestine samples by ether extraction followed by alumina column cleanup. The total yield was 90+/- 5%. No derivatization was necessary prior to GLC analysis.

HIPIN--a generic HIS/RIS-PACS interface based on clinical radiodiagnostic procedures.

Within the EurIPACS HIPIN topic a generic HIS/RIS-PACS interface will be designed, implemented and evaluated. It is generally agreed that integration with the HIS/RIS is essential for the acceptance of PACS in a clinical environment. An interface between HIS/RIS and PACS allows more efficient usage of both systems, better integration of data, better consistency checking on shared data and better security and error handling. Also the PACS performance is improved by using HIS/RIS information to steer the image migration within the PACS. In this paper the functional specifications of the interface are described. These specifications are based on descriptions of clinical radiodiagnostic procedures. The generic interface consists of a common part, and of site specific adapters. The common part is identical for all incarnations and performs message scheduling, processing and logging. The adapters are specific for each communication standard, e.g. ACR-NEMA or HL7, and for each hospital. The interface will be implemented at the radiology department of the Philipps University Hospital in Marburg (Germany) and at the orthopaedic and neuroradiology departments of the hospital of the Free University in Brussels (Belgium).