Advanced portable micro-SORS prototype coupled with SERDS for heritage science.

We investigate the subsurface composition of turbid materials at the micro scale by means of a portable non-invasive technique, micro-spatially offset Raman spectroscopy (micro-SORS), combined with shifted excitation Raman difference spectroscopy (SERDS). This combination enables the microscale layer analysis and allows to deal effectively with highly fluorescing samples as well as ambient light, all in a form of an in-house portable prototype device optimised for applications in heritage science. The instrument comprises ability to simultaneously collect multiple spectra by means of an optical fibre bundle, thus reducing the dead time and simplifying the ease of deployment of the technique. The performance of the synergy between micro-SORS and 785 nm SERDS dual-wavelength diode laser is demonstrated on a stratified mock-up painting samples including highly fluorescing painted layers. This instrumental approach could be ground-breaking in heritage science, due to the largely unmet need of analysing the molecular composition of subsurface of artworks non-invasively and in situ, and in the presence of fluorescent background and ambient light. Moreover, many other fields are expected to benefit from this technological advancement such as solar energy, forensic and food analytical areas.

[Radiation-induced cataract-an occult risk for urologists]. / Strahleninduzierter Katarakt ­ ein okkultes Berufsrisiko für Urologen.

BACKGROUND: Radiation-induced cataracts represent a relevant risk to people occupationally exposed to radiation. The annual limit dose for the eye lens was reduced to 20 mSv per year by German legislation (radiation protect law StrlSchG 2017; 2013/59/Euratom) based on recommendation of International Commission on Radiation Protection (2011 ICRP) to avoid radiation-induced cataracts. OBJECTIVES: Is there a risk of exceeding the annual limit dose for the eye lens in routine urological practice without special radiation protection for the head? METHODS: As part of a prospective, monocentric dosimetry study, of 542 different urological, fluoroscopically guided interventions, the eye lens dose was determined using a forehead dosimeter (thermo-luminescence dosemeter TLD, Chipstrate) over a period of 5 months. RESULTS: An average head dose of 0.05 mSv per intervention (max. 0.29 mSv) was found with an average dose area product of 485.33 Gy/cm2. Significant influencing factors for a higher dose were a higher patient body mass index (BMI), a longer operation time, and a higher dose area product. The level of experience of the surgeon showed no significant influence. DISCUSSION: With 400 procedures per year or an average of 2 procedures per working day, the critical annual limit value for the eye lenses or for the risk of radiation-induced cataract would be exceeded without special protective measures. CONCLUSION: Consistently effective radiation protection of the eye lens is essential for daily work in uroradiological interventions. This may require further technical developments.

[Characteristics and prevalence of painful coenesthesia in a pain clinic]. / Charakteristik und Prävalenz schmerzhafter Zönästhesien in einer Schmerzambulanz.

BACKGROUND: Coenesthesia, a rarely described symptom, is classified as schizophrenia according to ICD-10 and can occur independently of psychiatric diseases as a form of pain. The prevalence in chronic pain disorders is still unknown. The present study investigates the characteristics and psychological comorbidities of painful coenesthesia. MATERIALS AND METHODS: For the present study, all patients were pseudonymized and retrospectively analyzed qualitatively and quantitatively for existing coenesthesia. They were consecutively admitted, examined, and treated in the pain outpatient clinic of the University of Magdeburg over a five-year period (2013-2017). RESULTS: Of the 844 patients evaluated, 57 (6.7%) fulfilled the criteria of coenesthesia. The pain description may be rather bizarre if the patient is suffering from a psychiatric disorder, but it was also conspicuous by inappropriate localization for the pain description (tooth cramp instead of abdominal cramp). In our study, pain was mainly localized in the facial area (n = 35). Twenty-seven patients had no psychopathological abnormalities and 30 patients could be assigned an additional psychiatric diagnosis. In 23 patients, depression occurred as a psychological comorbidity. DISCUSSION AND CONCLUSION: Coenesthesia does not necessarily occur in connection or only with schizophrenia. Coenesthesia should be considered if the patient gives a bizarre description of pain, but also in common pain descriptions, such as burning, stabbing, cramping, or a feeling of pressure, if these are related to unusual locations (cramping tooth).

Importance of confirming the underlying diagnosis in patients with myocardial infarction and non-obstructive coronary arteries (MINOCA): a single-centre retrospective cohort study.

BACKGROUND: Many patients with myocardial infarction with non-obstructive coronary arteries (MINOCA) are discharged without a known aetiology for their clinical presentation. This study sought to assess the effect of this 'indeterminate MINOCA' diagnosis on the prevalence of recurrent cardiovascular events and presentations to the Cardiac Emergency Department (CED). METHODS: We retrospectively analysed all patients meeting the diagnostic MINOCA criteria presenting at a large secondary hospital between January 2017 and April 2019. PARTICIPANTS: Patients were divided into the (1) 'indeterminate MINOCA', or (2) 'MINOCA with diagnosis' group. The primary outcome was the occurrence of major adverse cardiac events (MACE) defined as the composite of all-cause mortality, non-fatal myocardial infarction, stroke and any revascularisation procedure. Secondary outcomes were all recurrent visits at the CED, and MACE including unplanned cardiac hospitalisation. RESULTS: In 62/198 (31.3%) MINOCA patients, a conclusive diagnosis was found (myocardial infarction, (peri)myocarditis, cardiomyopathy, or miscellaneous). MINOCA patients with a confirmed diagnosis were younger compared to those with an indeterminate diagnosis (56.7 vs. 62.3 years, p = 0.007), had higher maximum troponin-T [238 ng/L vs. 69 ng/L, p < 0.001] and creatine kinase (CK) levels [212U/L vs. 152U/L, p = 0.007], and presented more frequently with electrocardiographic signs of ischaemia (71.0% vs. 47.1%, p = 0.002). Indeterminate MINOCA patients more often showed recurrent CED presentations (36.8% vs. 22.6%, p = 0.048), however the occurrence of cardiovascular events was equal (8.8 vs. 8.1%, p = 0.86). Multivariable analysis showed that elevated levels of troponin-T and CK, ST-segment deviation on electrocardiography, reduced left ventricular ejection fraction, regional wall motion abnormalities, and performance of additional examination methods were independent predictors for finding the underlying MINOCA cause. CONCLUSIONS: Only in one-third of MINOCA patients a conclusive diagnosis for the acute presentation was identified. Recurrent CED visits were more often observed in the indeterminate MINOCA group, while the occurrence of cardiovascular events was similar across groups. TRIAL REGISTRATION: Retrospectively registered.

Sharing primary percutaneous coronary intervention care: first experiences with South Limburg ST-elevation myocardial infarction network.

BACKGROUND: In the region of South Limburg, the Netherlands, a shared ST-elevation myocardial infarction (STEMI) networking system (SLIM network) was implemented. During out-of-office hours, two percutaneous coronary intervention (PCI) centres-Maastricht University Medical Centre and Zuyderland Medical Centre-are supported by the same interventional cardiologist. The aim of this study was to analyse performance indicators within this network and to compare them with contemporary European Society of Cardiology guidelines. METHODS: Key time indicators for an all-comer STEMI population were registered by the emergency medical service and the PCI centres. The time measurements showed a non-Gaussian distribution; they are presented as median with 25th and 75th percentiles. RESULTS: Between 1 February 2018 and 31 March 2019, a total of 570 STEMI patients were admitted to the participating centres. The total system delay (from emergency call to needle time) was 65 min (53-77), with a prehospital system delay of 40 min (34-47) and a door-to-needle time of 22 min (15-34). Compared with in-office hours, out-of-office hours significantly lengthened system delays (55 (47-66) vs 70 min (62-81), p < 0.001), emergency medical service transport times (29 (24-34) vs 35 min (29-40), p < 0.001) and door-to-needle times (17 (14-26) vs 26 min (18-37), p < 0.001). CONCLUSIONS: With its effective patient pathway management, the SLIM network was able to meet the quality criteria set by contemporary European revascularisation guidelines.

[Combined vitrectomy-lensectomy surgery with bag-in-the-lens or lens in the bag implantation: comparison of final visual acuity]. / Chirurgie combinée vitrectomie et phakoéxérèse avec implantation bag-in-the-lens (BIL) ou de sac : comparaison de l'acuité visuelle finale.

INTRODUCTION: Combined vitrectomy-lensectomy surgery is a safe and effective procedure. Nevertheless, it is frequently complicated by posterior capsule opacification and the formation of posterior synechiae. These complications can be avoided by placing a "bag in the lens" (BIL) implant. The objective of this study is to compare the visual acuity gain (VA) after combined vitrectomy-lensectomy surgery between a group implanted with the BIL technique and a group with implantation in the bag (LIB). MATERIAL AND METHODS: We included in the study all vitrectomy-lensectomy procedures for epiretinal membrane and vitreomacular traction performed between May 2013 and July 2016 at the Hospital and University Center of Caen. We compared the VA gain between the BIL group and the LIB group six months after surgery. RESULTS: A total of 33 patients were included in the study, consisting of 28 eyes in the BIL group and 8 eyes in the LIB group. The mean VA gain in the BIL group was -0.52 LogMAR (P<0.0001) and -0.56 LogMAR (P=0.0047) for the LIB group. The difference between the two groups was not significant (P=0.74). CONCLUSION: The use of the BIL technique during vitrectomy-lensectomy allows visual recovery as good as implantation within the capsular bag. In addition, this implant has the advantage of significantly reducing the occurrence of posterior synechiae and preventing anterior and posterior capsular proliferation.

Ptchd1 deficiency induces excitatory synaptic and cognitive dysfunctions in mouse.

Synapse development and neuronal activity represent fundamental processes for the establishment of cognitive function. Structural organization as well as signalling pathways from receptor stimulation to gene expression regulation are mediated by synaptic activity and misregulated in neurodevelopmental disorders such as autism spectrum disorder (ASD) and intellectual disability (ID). Deleterious mutations in the PTCHD1 (Patched domain containing 1) gene have been described in male patients with X-linked ID and/or ASD. The structure of PTCHD1 protein is similar to the Patched (PTCH1) receptor; however, the cellular mechanisms and pathways associated with PTCHD1 in the developing brain are poorly determined. Here we show that PTCHD1 displays a C-terminal PDZ-binding motif that binds to the postsynaptic proteins PSD95 and SAP102. We also report that PTCHD1 is unable to rescue the canonical sonic hedgehog (SHH) pathway in cells depleted of PTCH1, suggesting that both proteins are involved in distinct cellular signalling pathways. We find that Ptchd1 deficiency in male mice (Ptchd1-/y) induces global changes in synaptic gene expression, affects the expression of the immediate-early expression genes Egr1 and Npas4 and finally impairs excitatory synaptic structure and neuronal excitatory activity in the hippocampus, leading to cognitive dysfunction, motor disabilities and hyperactivity. Thus our results support that PTCHD1 deficiency induces a neurodevelopmental disorder causing excitatory synaptic dysfunction.

Sclerotic scatter.

Sclerotic scatter involves the scattering of incident light by the limbal sclera followed by entry of part of the scattered light into the cornea, where some of the light travels through total internal reflection to the other side, where it scatters a second time in the limbal sclera. It is then visible in the form of a limbal scleral arc of light. Sclerotic scatter has been used for decades to spot and delineate corneal opacities, which disrupt and scatter the light travelling through total internal reflection. To implement the technique, the slit beam and the binoculars of the slit lamp should be dissociated so that the limbal sclera is illuminated, while the binoculars are centered on the cornea. The technique does not provide any information as to the depth of corneal opacities and therefore needs to be complemented by direct illumination. The second sclerotic scatter may also be used clinically, for instance for diode cycloablation, the posterior part of the arc of light projecting 0.5mm behind the scleral spur. This article aims to describe the phenomenon of sclerotic scatter, explaining how the slit-lamp should be set to use this technique, describing its clinical applications (in the opacified cornea and in the normal sclera), showing that the limbal scleral arc of light of sclerotic scatter may be seen under certain circumstances in daily life with the naked eye and, finally, explaining how the arc of light differs from peripheral light focusing ("Coroneo effect").

Treatment outcomes of recurrent respiratory papillomatosis : Retrospective analysis of juvenile and adult cases.

BACKGROUND: Recurrent respiratory papillomatosis (RRP) is a rare, chronic disease of viral etiology which is characterized by multiple, recurrent growth of papillomas in the aerodigestive tract. MATERIALS AND METHODS: The surgical outcomes and the recurrence rates of 106 patients with RRP of the larynx were analyzed. The patients were treated at the University of Magdeburg between 1983 and 2014. The surgical outcomes of conventional and laser surgery regarding time to relapse and complications were compared. In addition, the influence of the quadrivalent vaccine Gardasil® on the disease was analyzed in 10 patients. RESULTS: Children with RRP had a statistically significant greater number of surgeries throughout their lifetimes compared to adult patients. There were no differences between the adult (n = 79) and juvenile (n = 27) groups in the time to relapse and the number of surgeries/year. The time to relapse and number of procedures/year were not influenced by the various surgical methods. Complications after conventional and laser surgery were not statistically different between the two groups. Serious complications and the need for a tracheotomy were only reported after conventional surgery. In the 10 patients who were immunized with Gardasil®, a statistically significant lower number of surgeries/year after vaccination was achieved. CONCLUSION: RRP is a rare disease. Treatment requires experience and may be very difficult. The analysis of the course of the disease has shown that the treatment of choice is surgical excision with the CO2 laser combined with the quadrivalent or polyvalent vaccine. Consequent vaccination of both boys and girls has the potential to reduce the occurrence of RRP.

Sirolimus Ointment for Facial Angiofibromas in Individuals with Tuberous Sclerosis Complex.

Background. Facial angiofibromas affect most patients with tuberous sclerosis complex. They tend to progress, can cause recurrent bleeding and facial disfigurement, and have significant psychological effects. We reviewed the effectiveness and safety of topical sirolimus ointment 0.1%. We also assessed the effect of treatment on quality of life. Methods. We report our experience in using sirolimus ointment in 14 patients with TSC (9 children and 5 adults). The impact of sirolimus ointment was monitored with digital photography, dermatological review using a validated Facial Angiofibroma Severity Index (FASI), and quality of life assessments using the questionnaires PedsQL for children and SF36 for adults. Results. The FASI scores were improved in 12/14 cases after six months' treatment, and improvement was more likely in children (median FASI scores of improvement after treatment were 3 points for children and 1 for adults). Proxy-reported PedsQL scores for the total psychosocial domain improved significantly in the children in the cohort with treatment. Conclusions. Sirolimus ointment 0.1% administered once a day was effective in treating facial angiofibromas. It appears to be safe and well tolerated and to have a positive impact on patients' quality of life. It appeared to be most beneficial when started in childhood.

Novel 9 amino acid in-frame deletion in the NTRK1 tyrosine kinase domain in a patient with congenital insensitivity to pain with anhydrosis.

Schematic presentation of NTRK1 protein structure. Variants identified in this study are shown in red and previously reported variants associated with CIPA are shown in black (LRM, leucine rich motif; Ig, immunoglobulin-like domain; TM, transmembrane domain; TK, tyrosine kinase domain).

[Treatment outcomes of recurrent respiratory papillomatosis : Retrospective analysis of juvenile and adult cases. German version]. / Behandlungsergebnisse bei rezidivierender respiratorischer Papillomatose : Retrospektive Analyse juveniler und adulter Fälle.

BACKGROUND: Recurrent respiratory papillomatosis (RRP) is a rare, chronic disease of viral etiology which is characterized by multiple, recurrent growth of papillomas in the aerodigestive tract. MATERIALS AND METHODS: The surgical outcomes and the recurrence rates of 106 patients with RRP of the larynx were analyzed. The patients were treated at the University of Magdeburg between 1983 and 2014. The surgical outcomes of conventional and laser surgery regarding time to relapse and complications were compared. In addition, the influence of the quadrivalent vaccine Gardasil® on the disease was analyzed in 10 patients. RESULTS: Children with RRP had a statistically significant greater number of surgeries throughout their lifetimes compared to adult patients. There were no differences between the adult (n = 79) and juvenile (n = 27) groups in the time to relapse and the number of surgeries/year. The time to relapse and number of procedures/year were not influenced by the various surgical methods. Complications after conventional and laser surgery were not statistically different between the two groups. Serious complications and the need for a tracheotomy were only reported after conventional surgery. In the 10 patients who were immunized with Gardasil®, a statistically significant lower number of surgeries/year after vaccination was achieved. CONCLUSION: RRP is a rare disease. Treatment requires experience and may be very difficult. The analysis of the course of the disease has shown that the treatment of choice is surgical excision with the CO2 laser combined with the quadrivalent or polyvalent vaccine. Consequent vaccination of both boys and girls has the potential to reduce the occurrence of RRP.

Caregiver's perception of epilepsy treatment, quality of life and comorbidities in an international cohort of CDKL5 patients.

OBJECTIVE: CDKL5 is a genetic condition associated with drug-resistant epilepsy and intellectual disability. There is limited information on its natural history. We investigated the natural history, complications, and the effectiveness of current treatment strategies. METHODS: This study was conducted in conjunction with the CDKL5-UK Charity, with patients recruited from the USA and Europe. Online questionnaires were completed by parents/carers and included information relating to demographics, growth, development, epilepsy, comorbid conditions, and efficacy and side effects of antiepileptic treatments. RESULTS: Thirty-nine of the 44 patients were female. Median age was five years (range five months to 31 years), and all had a history of epilepsy. All patients had developmental delay, with 4/21 able to run and 4/22 able to climb. Gastrointestinal problems were reported in 31/43. Cardiac arrhythmia was seen in 11/29. Over one-quarter of the patients had tried ten or more antiepileptic medications. Vigabatrin was reportedly the most effective AED (antiepileptic drug) in 12/23; clobazam (most effective in 6/14); sodium valproate (most effective in 5/27), and levetiracetam (most effective in 3/27). VNS (Vagal Nerve Stimulator) was reported to be effective in 9/12. One year after VNS insertion, 9/12 reported improved (QoL), and there were improvements in mood, school achievement and concentration in (9/11). The ketogenic diet was considered effective and to have improved QoL in (12/23). CONCLUSION: Vigabatrin appears to be more effective than other AEDs. VNS and ketogenic diet are also relatively effective. Gastrointestinal and cardiovascular system complications are common. The results may help to guide management of epilepsy in CDKL5. It highlights a possible link between CDKL5 and potentially treatable life-threatening complications such as cardiac arrhythmia. More research in this area may help us develop a more systematic approach to treating these patients. HIPPOKRATIA 2017, 21(3): 130-135.

[Pain therapy in in-patients with cancer. Effects of a manual-based approach as guideline for pain-consulting service at a university hospital]. / Stationäre medikamentöse Schmerztherapie bei Tumorpatienten: Effekte einer manualisierten Leitlinie für den Konsildienst eines Universitätsklinikums.

BACKGROUND: Appropriate medication is an important and substantial part in the therapy of tumor-induced pain. OBJECTIVE: The objective of this study was to investigate the efficiency of anaesthesiology-based consultant service characterizing the quality of this type of treatment in daily clinical practice of a university hospital, i. e., in the patient profile of a tertiary center (study design: systematic clinical, unicenter observational study reflecting clinical practice and study-based control of therapeutic care quality). METHODS: In the course of consulting function with regard to pain care on the single wards a considerable portion of cancer patients are recieving drugs. For most patients such care comprises several consultations and subsequently initiated treatment modifications. The consulting function ends if the patients feel free of pain or report a substantial improvement. From 1/1/2010 to 12/31/2012 detailed information on the drug therapy applied prior to, during and after the consultation was prospectively documented.This data was retrospectively evaluated as "pre-vs.-post" comparison (Chi-squared test, Fisher's exact test and McNemar's test), in particular, focussing on the quality of pain medication using the WHO index as well as pain intensity obtained by means of the visual analogue scale (VAS). RESULTS: In total, 375 in-patients were treated. The modified pain medication by the anesthesiological consultant service led to a significant increase (p < 0.001; Wilcoxon's test) in the mean WHO index from 6.37 (SD, 1.83) to 8.43 (SD, 1.47). Furthermore, a reduction of VAS from 5.00 (SD, 2.39) to 2.64 (SD, 1.64) was noted (p < 0.001; Wilcoxon's test). CONCLUSION: The consequent application of established guidelines (according to WHO scheme) and the WHO index leads to a qualitative and measurable improvement of drug therapy for cancer-related pain.

[Are the ophthalmology items of the French health record realistic?]. / Les items d'ophtalmologie du carnet de santé sont-ils réalistes ?

PURPOSE: Since 2006, French general practitioners have had to carry out an ophthalmological screening for children, using a methodology contained within the child's health record (carnet de santé). The purpose of this screening is to allow an early diagnosis of sight-threatening as well as potentially life-threatening conditions (e.g., retinoblastoma). The aim of the present study was to evaluate the theoretical knowledge and the clinical skills of practitioners implementing the screening, with regards to two specific items of the health record screening schedule, by means of a questionnaire. MATERIAL AND METHODS: From December 2013 to September 2014, 42 practitioners (16 general practitioners, 14 pediatricians, 12 resident physicians) from four distinct locations in Normandy, France, were questioned using a questionnaire on two ophthalmological items of the French health record: one on red reflex and one on eyeball size. RESULTS: Only four of 42 practitioners provided correct answers on the red reflex color. Thirty of 42 practitioners reported difficulties performing the red reflex test, mostly because they did not know the normality criteria and the purpose served by this test. Thirty-three of 42 practitioners declared that they found it difficult to know whether or not eyeball sizes were normal, mostly because they were unaware of the normality criteria. None of the practitioners provided the correct (to within ± 1 mm) mean horizontal cornea diameter in full-term newborns and adults. CONCLUSION: This study demonstrates that there is poor theoretical knowledge and a low level of clinical skill for practitioners performing the ophthalmological screening based on the French health record schedule. This schedule therefore does not seem to meet its purpose.

Beyond-the-edge proliferation after relaxing retinectomy.

PURPOSE: To report beyond-the-edge proliferation (BTEP) after relaxing retinectomies (RR) i.e. fibrous sheets stretched between the RR edge and the far periphery; to evaluate the tractional potential and report the long-term course of BTEP. METHODS: Retrospective review of the medical records of 83 patients having undergone a RR between January 2009 and December 2014 to identify patients with BTEP. RESULTS: Six patients aged 31 to 76 were identified. Retinectomy had been performed for traumatic retinal incarceration in one case and anterior PVR in 5 cases. BTEP occurred within weeks of the RR (earliest: 5 weeks). It was discovered intraoperatively in two patients with silicone oil tamponade, at 7 weeks and 6 months respectively after RR. It recurred over a few months after excision in 5 patients, causing inferior tractional retinoschisis in 4 patients and inferior tractional retinal detachment in two patients. CONCLUSIONS: BTEP is an unusual form of proliferative vitreoretinopathy developing despite the absence of the usual vitreo-retinal support (excised during RR), probably through compartmentalization and cell migration along the inferior interface between silicone oil or gas and the aqueous humour. BTEP can cause serious retinal traction, develops over weeks after the RR and recurs frequently a few months after excision.

[Neurotrophic keratitis after vitrectomy and circumferential endophotocoagulation for retinal detachment]. / Kératite neurotrophique après chirurgie du décollement de rétine par vitrectomie et endophotocoagulation circonférentielle.

INTRODUCTION: Circumferential (360°) endophotocoagulation is frequently implemented during vitrectomies for retinal detachment. This photocoagulation may result in neurotrophic keratitis by damaging the ciliary nerves in the suprachoroidal space on their way to the pupil. We report a series of 4 cases of neurotrophic keratitis following a circumferential endophotocoagulation. PATIENTS AND METHODS: A retrospective observational case series of 4 non-diabetic patients having presented with a neurotrophic keratitis following a retinal detachment treated with vitrectomy and circumferential endophotocoagulation (532 nm) at Caen University Hospital. We report the various forms of corneal lesions and the diagnostic criteria allowing for the diagnosis of neurotrophic keratitis. DISCUSSION: Neurotrophic keratitis is caused by lesions occurring at various levels of corneal innervation. Endophotocoagulation may cause a neurotrophic keratitis by damaging the short and long ciliary nerves on their way to the pupil in the suprachoroidal space. The sequelae of this condition can limit visual recovery. Hence, it is probably advisable to screen for corneal anesthesia or severe hypesthesia following a retinal detachment treated with vitrectomy and circumferential endophotocoagulation and to implement prophylactic treatment (intensive lubricant therapy; preservative-free eye drops) if needed. CONCLUSION: The risk of neurotrophic keratitis should be weighed against the dose of laser retinopexy necessary and sufficient to obtain a sustained retinal reattachment. If circumferential endophotocoagulation is implemented, it is probably sensible to monitor corneal sensitivity and to adapt postoperative treatment if necessary.