scholarly journals Vogt-Koyanagi-Harada syndrome: Experience in a Belgian university hospital and review of the literature

2017 ◽  
Vol 4 (2) ◽  
pp. 53
Author(s):  
Liza Sels ◽  
Joachim Van Calster ◽  
Steven Vanderschueren ◽  
Liesbet Henckaerts

Introduction: The Vogt-Koyanagi-Harada syndrome (VKHS) is rare in Europe. Bilateral panuveitis is the defining characteristic, but the disease can affect other tissues containing melanin, such as the inner ear, the meninges, and the skin. Therefore, patients may present not only to ophthalmologists, but also to internists, dermatologists, ENT-physicians and neurologists. Early and aggressive immunosuppressive treatment is necessary in order to prevent permanent visual loss or systemic complications.Objective: We retrospectively studied 12 patients diagnosed and treated in a specialized uveitis clinic in Leuven (Belgium) between 2005 and 2013, and we compared our data with literature.Results:  The most common extra-ocular manifestations were neurological, with headache (100%) and lymphocytic meningitis (75%) as main findings. Tinnitus was present in 50%, and vertigo in 42%. Vitiligo was the most frequent dermatological manifestation and present in 42%. Uveitis recurred in 67% of patients with 1 to 4 relapses per patient and a median time to recurrence of 6 months (range 1-11). Normal visual acuity was seen in 75% of patients at the end of follow-up (5 to 60 months), but 3 patients had at least some degree of permanent visual impairment in at least one eye.Conclusion: VKHS is a rare, multisystem disorder, characterized by bilateral uveitis and variable neurologic, auditory and skin symptoms. A lumbar puncture increases the diagnostic yield. A fast diagnosis allows timely initiation of adequate therapy, and If treated correctly, VKHS has a favorable prognosis. It is unclear if initial intravenous therapy improves frequency and time to remission.

2018 ◽  
Vol 49 (03) ◽  
pp. 193-199 ◽  
Author(s):  
Daniel Ebrahimi-Fakhari ◽  
Ludwig Gortner ◽  
Martin Poryo ◽  
Michael Zemlin ◽  
Alfons Macaya-Ruiz ◽  
...  

AbstractTuberous sclerosis complex (TSC) is a genetic disease with a significant morbidity and mortality. We conducted a retrospective analysis of two cohorts (Vall d'Hebron University Hospital [HVH], Barcelona, Spain, 1982–2015, and at Saarland University Medical Center [UKS], Homburg, Germany, 1998–2015) to assess prevalence and treatment of TSC associated manifestations and to evaluate if the follow-up was in line with published recommendations. This was considered if more than 15% of patients did not receive adequate examination with regard to potential organ involvement. A definite diagnosis was made in 52 patients (96%), and a possible diagnosis was made in 2 patients (4%). Thirty-four (63%) patients were from HVH and 20 (37%) from UKS. Median age at first presentation was 6 months (interquartile range: 0–38 months), and median time of follow-up was 6 years (interquartile range: 2–13 years). Clinical symptoms that led to a diagnosis of TSC were cardiac rhabdomyoma (22/54), epilepsy (20/54), and cutaneous manifestations (4/54). Assessment of neuropsychiatric, renal, and ocular manifestations was inadequate in both hospitals, whereas cutaneous manifestation was inadequate at UKS only. Our data demonstrate insufficient examinations in a substantial number of TSC patients with regard to neuropsychiatric, renal, ocular, and cutaneous manifestations. The recently published guidelines may prove valuable in establishing a more comprehensive approach.


BMJ Open ◽  
2021 ◽  
Vol 11 (11) ◽  
pp. e049559
Author(s):  
Lilja Thorgeirsdottir ◽  
Malin Andersson ◽  
Ove Karlsson ◽  
Sven-Egron Thörn ◽  
Jonatan Oras ◽  
...  

IntroductionPre-eclampsia, a multisystem disorder in pregnancy, is one of the most common causes of maternal morbidity and mortality worldwide. However, we lack methods for objective assessment of organ function in pre-eclampsia and predictors of organ impairment during and after pre-eclampsia. The women’s and their partners’ experiences of pre-eclampsia have not been studied in detail. To phenotype different subtypes of the disorder is of importance for prediction, prevention, surveillance, treatment and follow-up of pre-eclampsia.The aim of this study is to set up a multicentre database and biobank for pre-eclampsia in order to contribute to a safer and more individualised treatment and care.Methods and analysisThis is a multicentre cohort study. Prospectively recruited pregnant women ≥18 years, diagnosed with pre-eclampsia presenting at Sahlgrenska University Hospital, Uppsala University Hospital and at Södra Älvsborgs Hospital, Sweden, as well as normotensive controls are eligible for participation. At inclusion and at 1-year follow-up, the participants donate biosamples that are stored in a biobank and they are also asked to participate in various organ-specific evaluations. In addition, questionnaires and interviews regarding the women’s and partner’s experiences are distributed at follow-up.Ethics and disseminationBy creating a database and biobank, we will provide the means to explore the disorder in a broader sense and allow clinical and laboratory discoveries that can be translated to clinical trials aiming at improved care of women with pre-eclampsia. Further, to evaluate experiences and the psychological impact of being affected by pre-eclampsia can improve the care of pregnant women and their partners. In case of incidental pathological findings during examinations performed, they will be handled in accordance with clinical routine. Data are stored in a secure online database. Biobank samples are identified through the women’s personal identification number and pseudonymised after identification in the biobank before analysis.This study was approved by the regional ethical review board in Gothenburg on 28 December 2018 (approval number 955-18) and by the Swedish Ethical Review Authority on 27 February 2019 (approval number 2019-00309).Results from the study will be published in international peer-reviewed journals.Trial registration numberISRCTN13060768


2017 ◽  
Vol 4 (suppl_1) ◽  
pp. S88-S88
Author(s):  
Tohru Takata ◽  
Yoshinobu Yoshimura ◽  
Yumiko Obata ◽  
Atsushi Togawa ◽  
Yasushi Takamatsu

Abstract Background Ocular candidiasis is a major complication of candidemia; however, many remains unknown for the incidence, risk factors, and outcome of eye involvement. Methods We retrospectively reviewed the medical records and obtained information related to fungal infection and its management, and visual outcome at Fukuoka University Hospital from 2000 to 2016. Results Of 143 patients with candidemia for whom an ophthalmology consult was requested, 26 had findings consistent with the diagnosis of ocular candidiasis. Patients with ocular candidiasis were mostly infected with Candida albicans (n = 20), followed by C. glabrata (n = 4), and C. tropicalis (n = 2). In contrast, only one patient infected with C. parapsilosis had ocular involvement although the number of the patients with C. parapsilosis candidemia was second the most among candidemia. No difference was seen for the β-d-glucan in patients with or without ocular candidiasis (128.6 vs. 106.1, P = 0.654). All of the isolates other than C. glabrata were susceptible to fluconazole. In all of 23 patients with existing central venous cathers, CVCs were removed after the diagnosis of candidemia. Four-week mortality rate in patients with ocular candidiasis was 16.7% (three of 18 patients) which was not significantly different from that in patients without ocular manifestations. All treated patients were confirmed for clearance of candidemia, received systemic antifungals, and improved for visual outcome or remained stable, and no patients complicated visual loss without surgical treatment. Therapy with micafungin or caspofungin followed by fluconazole (12 patients) was successful in all patients. Conclusion Ocular involvement occurred in 18% of patients with candidemia, and treatment with echinocandins followed by fluconazole was successful in most cases with follow-up. Disclosures T. Takata, Taisho Toyama Pharma: Speaker’s Bureau, Speaker honorarium


2019 ◽  
Author(s):  
M Stättermayer ◽  
F Riedl ◽  
S Bernhofer ◽  
A Stättermayer ◽  
A Mayer ◽  
...  

2014 ◽  
Vol 23 (2) ◽  
pp. 179-185 ◽  
Author(s):  
Suvadip Chatterjee ◽  
Kofi W. Oppong ◽  
John S. Scott ◽  
Dave E. Jones ◽  
Richard M. Charnley ◽  
...  

Background & Aims: Autoimmune pancreatitis (AIP) is a fibroinflammatory condition affecting the pancreas and could present as a multisystem disorder. Diagnosis and management can pose a diagnostic challenge in certain groups of patients. We report our experience of managing this condition in a tertiary pancreaticobiliary centre in the North East of England.Methods: Patients were identified from a prospectively maintained database of patients diagnosed with AIP between 2005 and 2013. Diagnosis of definite/probable AIP was based on the revised HISORt criteria. When indicated, patients were treated with steroids and relapses were treated with azathioprine. All patients have been followed up to date.Results: Twenty-two patients were diagnosed with AIP during this period. All patients had pancreatic protocol CT performed while some patients had either MR or EUS as part of the work up. Fourteen out of 22 (64%) had an elevated IgG4 level (mean: 10.9 g/L; range 3.4 - 31 g/L). Four (18%) patients underwent surgery. Extrapancreatic involvement was seen in 15 (68%) patients, with biliary involvement being the commonest. Nineteen (86%) were treated with steroids and five (23%) required further immunosuppression for treatment of relapses. The mean follow up period was 36.94 months (range 7 - 94).Conclusion: Autoimmune pancreatitis is being increasingly recognized in the British population. Extrapancreatic involvement, particularly extrahepatic biliary involvement seems to be a frequent feature.Diagnosis should be based on accepted criteria as this significantly reduces the chances of overlooking malignancy. Awareness of this relatively rare condition and a multi-disciplinary team approach will help us to diagnose and treat this condition more efiectively thereby reducing unnecessary interventions.


Author(s):  
Riccardo Balestri ◽  
Giulia Odorici ◽  
Annalisa Patrizi ◽  
Salvatore D. Infusino ◽  
Michela Magnano ◽  
...  

2017 ◽  
pp. 50-55
Author(s):  
Duc Luu Ngo ◽  
Tu The Nguyen ◽  
Manh Hung Ho ◽  
Thanh Thai Le

Background: This study aims to survey some clinical features, indications and results of tracheotomy at Hue Central Hospital and Hue University Hospital. Patients and method: Studying on 77 patients who underwent tracheotomy at all of departments and designed as an prospective, descriptive and interventional study. Results: Male-female ratio was 4/1. Mean age was 49 years. Career: farmer 44.2%, worker 27.2%, officials 14.3%, student 7.8%, other jobs 6.5%. Respiratory condition before tracheotomy: underwent intubation 62.3%, didn’t undergo intubation 37.7%. Period of stay of endotracheal tube: 1-5 days 29.2%, 6-14 days 52.1%, >14 days 18.7%. Levels of dyspnea before tracheotomy: level I 41.4%, level II 48.3%, level III 0%, 10.3% of cases didn’t have dyspnea. Twenty cases (26%) were performed as an emergency while fifty seven (74%) as elective produces. Classic indications (37.7%) and modern indications (62.3%). On the bases of the site, we divided tracheostomy into three groups: high (0%), mid (25.3%) and low (74.7%). During follow-up, 44 complications occurred in 29 patients (37.7%). Tracheobronchitis 14.3%, tube obstruction 13%, subcutaneous empysema 10.4%, hemorrhage 5%, diffcult decannulation 5.2%, tube displacement 3.9%, canule watery past 2.6%, wound infection 1.3%. The final result after tracheotomy 3 months: there are 33 patients (42.9%) were successfully decannulated. In the 33 patients who were successfully decannulated: the duration of tracheotomy ranged from 1 day to 90 days, beautiful scar (51.5%), medium scar (36.4%), bad scar (12.1%). Conclusions: In tracheotomy male were more than female, adult were more than children. The main indication was morden indication. Tracheobronchitis and tube obstruction were more common than other complications. Key words: Tracheotomy


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