scholarly journals Unique clinical spectrum with distinguishing diagnostic features in Vogt-Koyanagi-Harada syndrome

2019 ◽  
Vol 12 (12) ◽  
pp. e231397
Author(s):  
Mamoona Sultan ◽  
Adeena Khan ◽  
Syed Shahid Habib ◽  
Dheyab Abdulsalam
Keyword(s):  
Nerve Conduction ◽  
Immunosuppressive Drug ◽  
Diagnostic Features ◽  
Cutaneous Manifestations ◽  
Ophthalmic Manifestations ◽  
Systemic Corticosteroids ◽  
Appropriate Treatment ◽  
Ophthalmic Imaging ◽  
First Time

A 36-year-old ulcerative colitis male patient on treatment for 7 years was referred to dermatology with resistant alopecia universalis and hypopigmented patches on limbs for 5 months. During this time he also reported to ophthalmology with acute bilateral decreased vision for 5 days. His examination revealed hyperaemic discs, multifocal retinal detachments and choroidal granulomas. Taking into account the revised diagnostic criteria, atypical course of disease in the form of early cutaneous presentation followed by ophthalmic manifestations was attributed to Vogt-Koyanagi-Harada syndrome (VKHS) which was supported by relevant investigations including ophthalmic imaging, MRI and nerve conduction studies. Subclinical nerve conduction abnormalities and white matter demyelination were also seen for the first time in a patient of VKHS. Appropriate treatment was required to prevent visual complications; therefore, systemic corticosteroids with steroid sparing immunosuppressive drug therapy showed significant improvement in vision on follow-up. Cutaneous manifestations were resilient to the entire regimen.

scholarly journals PSORIAZINE ARTROPATIJA SERGANČIŲJŲ, HOSPITALIZUOTŲ 2013 METAIS DERMATOVENEROLOGIJOS CENTRE, ANALIZĖ

2015 ◽  
Vol 25 (1) ◽  
pp. 78-84
Author(s):  
Dalia Pacevičiūtė ◽  
Vaitiekus Julius Lavinskas ◽  
Tatjana Orlovskytė ◽  
Jonas Lauraitis ◽  
Matilda Bylaitė Bučinskienė
Keyword(s):  
Psoriatic Arthritis ◽  
Severe Psoriasis ◽  
Age Group ◽  
Correct Treatment ◽  
Systemic Corticosteroids ◽  
Appropriate Treatment ◽  
Hospitalised Patients ◽  
Working Age ◽  
Vilnius University ◽  
First Time

Psoriatic arthropaty - chronic, autoimmunive, progressing and 30% of all cases deformating joints severe psoriasis illness, that causes disability. The aim of the research: To analyse the patients with psoriatic arthritis who were hospitalised in Vilnius University Santariskiu Clinics Centre of Dermatoveneorology during the year of 2013. Methodology: Retrospective analysis was performed for patients with psoriasis and psoriatic arthritis who were hospitalised in VUH SC Centre of Dermatovenerology during the year of 2013. We have evaluated patients diagnosed with psoriasis and psoriatic arthritis, their sex, age, lenght of the illness, age of diagnosing psoriasis, severity according PASI, joint infingements, complaints, treatment, disability and level of work abilities. Data analysis was made using ‚‘Excel‘‘ statistical software. Results: Totally 651 patients were treated in Centre of Dermatovenereology during the year of 2013. 291 (45%) patients were hospitalised with diagnosis of psoriasis, 121 (42%) of them were also diagnosed with psoriatic arthritis. At the age group to 25 years old 29 (10%) patients were diagnosed with psoriatic arthritis. There were 182 (63%) patients at the age group between 25 and 60 years old with psoriatic arthritis. Over 60 years old there were 79 (27%) patients suffering from psoriatic arthritis. Average lenght of psoriasis in hospitalised patients was 20 years, average age of patients when first time diagnosed with psoriasis was 29 years. The average meaning of PASI was 22. Patients diagnosed with psoriatic arthritis most oftenly complained of these joint pains: ankles and knees - 61 patient (52%), palm joints - 56 (50%), feet - 39 (33%), wrists - 28 (24%), elbows and spine - 27 (23%), shoulders - 21 (18%), hips - 18 (15%), palm fingers - 12 (10%). Systemic treatment was applied totally for 82 (28%) hospitalised patients: 54 (19%) were prescribed methotrexate, 26 (9%) patients - systemic corticosteroids, 2 (1%) patients - biological therapy with etanercept. 186 (77%) patients with severe psoriasis are hospitalised in average 1,1 time per year, remision remains 0,5 to 1 year. At least 25% hospitalised with psoriasis or psoriatic arthritis have disability, their work posibilities are reduced to 50%. Conclusions: According to research during the year of 2013 there were hospitalised 121 patients with diagnosis of psoriatic arthritis. Even 30% of them had joint infringements and severe course of the illness that caused disability. Most of them psoriatic arthritis occured at the young and working age. Evaluating severity of the psoriasis we discovered that 69% patients hospitalised in 2013 had PASI higher than 15. In Lithuania there is possible to get appropriate treatment for psoriasis and psoriatic arthritis. It is aproved that applying correct treatment patients can reduce symptoms and maintain their physical abbilities.

scholarly journals Idiopathic pulmonary fibrosis and sleep disorders: no longer strangers in the night

2015 ◽  
Vol 24 (136) ◽  
pp. 327-339 ◽  
Author(s):  
Sophia Schiza ◽  
Charalampos Mermigkis ◽  
George A. Margaritopoulos ◽  
Zoi Daniil ◽  
Sergio Harari ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Positive Airway Pressure ◽  
Sleep Apnoea ◽  
New Drugs ◽  
Pressure Therapy ◽  
Appropriate Treatment ◽  
Obstructive Sleep ◽  
First Time

The prevalence of obstructive sleep apnoea (OSA) is continuously increasing in patients with idiopathic pulmonary fibrosis (IPF) and, for the first time, the recent IPF guidelines recognise OSA as an important associated comorbidity that can affect patient's survival. Thus, it becomes conceivable that clinicians should refer patients with newly diagnosed IPF to sleep centres for the diagnosis and treatment of OSA as well as for addressing issues regarding the reduced compliance of patients with continuous positive airway pressure therapy. The discovery of biomarkers common to both disorders may help early diagnosis, institution of the most appropriate treatment and follow-up of patients. Better understanding of epigenetic changes may provide useful information about pathogenesis and, possibly, development of new drugs for a dismal disease like IPF.

Psychological Resilience Provides No Independent Protection From Suicidal Risk

Crisis ◽  
2016 ◽  
Vol 37 (2) ◽  
pp. 130-139 ◽  
Author(s):  
Danica W. Y. Liu ◽  
A. Kate Fairweather-Schmidt ◽  
Richard Burns ◽  
Rachel M. Roberts ◽  
Kaarin J. Anstey
Keyword(s):  
Risk Factors ◽  
Well Being ◽  
Current Status ◽  
Psychological Resilience ◽  
Cross Sectional ◽  
Life Project ◽  
Resilience Factors ◽  
First Time

Abstract. Background: Little is known about the role of resilience in the likelihood of suicidal ideation (SI) over time. Aims: We examined the association between resilience and SI in a young-adult cohort over 4 years. Our objectives were to determine whether resilience was associated with SI at follow-up or, conversely, whether SI was associated with lowered resilience at follow-up. Method: Participants were selected from the Personality and Total Health (PATH) Through Life Project from Canberra and Queanbeyan, Australia, aged 28–32 years at the first time point and 32–36 at the second. Multinomial, linear, and binary regression analyses explored the association between resilience and SI over two time points. Models were adjusted for suicidality risk factors. Results: While unadjusted analyses identified associations between resilience and SI, these effects were fully explained by the inclusion of other suicidality risk factors. Conclusion: Despite strong cross-sectional associations, resilience and SI appear to be unrelated in a longitudinal context, once risk/resilience factors are controlled for. As independent indicators of psychological well-being, suicidality and resilience are essential if current status is to be captured. However, the addition of other factors (e.g., support, mastery) makes this association tenuous. Consequently, resilience per se may not be protective of SI.

RESULTS OF INTRALESIONAL TRIAMCINOLONE ACETONIDE FOR CHALAZION TREATMENT

2011 ◽  
pp. 100-104
Author(s):  
Thi Thu Nguyen ◽  
Viet Hien Vo ◽  
Thi Em Do
Keyword(s):  
Success Rate ◽  
Key Words ◽  
Triamcinolone Acetonide ◽  
Lower Eyelid ◽  
Upper Eyelid ◽  
Interventional Trial ◽  
The Mean ◽  
First Time

The study use intralesional triamcinolone acetonide injection proceduce for chalazion treatment.1. Objectives: To evaluate results of intralesional triamcinolone acetonide injection for chalazion treatment. 2. Method: This noncomparative prospective interventional trial included 72 chalazions of 61 patients. 3. Results: 61 patients (72 chalazions) with 19 males (31.1%) và 42 females (68.9%), the mean age was 24 ± 9,78 years. 31.1% patients was the first time chalazion and 68.9% patients was more than one times chalazion including 78.6% patients was recurrent at the first position and 21.4% patients occur at new position. 72 chalazions with 16 (22.2%) chalazions was treated before and 56 (77.8%) chalazions wasn’t done that. 72 chalazions with 49 chalazions (68.1%) are local in upper eyelid and 23 chalazions (31.9%) are local in lower eyelid. The mean of chalazion diameter is 6.99 ± 3.03mm. Intralesional triamcinolone acetonide is injected to treat 72 chalazions with 16 (22.2%) chalazions are injected through the route of skin and 56 (77.8%) chalazions are injected through the route of conjunctiva. After 2 weeks follow-up, the success rate was 93.1% and 6.9% failed. 4. Conclusion: intralesional triamcinolone acetonide injection for chalazion treatment is really effective. Key words: chalazion, intralesional triamcinolone acetonide.

scholarly journals Laparoscopic Resection of Renal Capsular Endometriosis in a Woman with Menstrual-Related Flank Pain: Case Report

2021 ◽  
Vol 27 (1) ◽  
Author(s):  
Abbas Basiri ◽  
Iman Ghanaat ◽  
Hamidreza Akbari Gilani
Keyword(s):  
Flank Pain ◽  
Pathological Examination ◽  
Histologic Evaluation ◽  
Normal Renal Parenchyma ◽  
Perinephric Space ◽  
Superior Portion ◽  
The Right ◽  
First Time ◽  
Laparoscopic Removal

Abstract Background Although involvement of the urinary system is not uncommon, endometriosis in the kidneys is rare. To date, laparoscopic partial nephrectomy has been the preferred approach for managing renal endometriosis. Here, we report for the first time the results of laparoscopic removal of a renal capsular endometriosis in a malrotated kidney in an attempt to save the whole kidney parenchyma, in terms of feasibility and safety. Case presentation A 37-year-old female presented with periodic right flank pain associated with her menstrual cycle. On imaging, a malrotated right kidney and a hypodense irregular-shaped lesion measuring 30 * 20 * 15 mm were seen in the superior portion of the right perinephric space. Histologic evaluation of the ultrasound-guided biopsy was consistent with renal capsular endometriosis. The patient underwent laparoscopic surgery to remove the capsular mass while preserving the normal renal parenchyma. Pathological examination of the biopsy obtained during surgery was in favor of renal endometriosis. At 6-month follow-up, the patient’s pain had completely disappeared and no complications had occurred. In addition, imaging did not show any remarkable recurrence. Conclusion Renal endometriosis should be strongly considered as a differential diagnosis in female patients with a renal capsular mass and exacerbation of flank pain during menstruation. Based on our experience, with preoperative needle biopsy and clearing the pathology, laparoscopic removal of the mass in spite of renal anatomic abnormality is feasible and safe and thus could be considered as a possible treatment option.

Progressive Leukodystrophy-Like Demyelinating Syndromes with MOG-Antibodies in Children: A Rare Under-Recognized Phenotype

2021 ◽  
Author(s):  
Elise Yazbeck ◽  
Hélène Maurey ◽  
Carole Leroy ◽  
Philippe Horellou ◽  
Silvia Napuri ◽  
...  
Keyword(s):  
Brain Magnetic Resonance Imaging ◽  
Brain Biopsy ◽  
Active Immunotherapy ◽  
Oligoclonal Bands ◽  
Second Child ◽  
Fluid Analysis ◽  
Appropriate Treatment ◽  
Neurological Prognosis ◽  
Mog Antibodies

AbstractAcquired demyelinating syndromes (ADS) are frequently associated with myelin oligodendrocytes glycoprotein (MOG) antibodies in children. Clinical phenotypes are heterogeneous and may delay the diagnosis, especially when they relapse and are atypical, mimicking diseases such as multiple sclerosis or neuromyelitis optica spectrum disorders . Here, we describe two children: one with a progressive cognitive and behavioral deterioration with seizures after only one relapse and the other with similar clinical impairments associated with multiple relapses. Brain magnetic resonance imaging revealed a subsequent progressive leukodystrophy-like lesion with diffuse bilateral white matter injuries in both patients. Cerebrospinal fluid analysis showed pleiocytosis, increased level of proteins with no oligoclonal bands. Metabolic and inflammatory blood markers were all negative. Brain biopsy was performed in the second child and nonspecific inflammatory lesions with no argument for histiocytosis or tumor were observed. Clinical and radiological stabilization were obtained after active immunotherapy. Retrospective analysis of anti-MOG antibodies in these two children was positive at the earlier stage of the disease and turned negative after treatment and during follow-up. Leukodystrophy-like ADS with anti-MOG-antibodies may display distinct progressive phenotype and have a severe neurological prognosis. Early diagnosis and appropriate treatment may improve outcome in these children.

scholarly journals Programme for Harmonization to the International Scale in Latin America for BCR-ABL1 quantification in CML patients: findings and recommendations

2020 ◽  
Vol 58 (12) ◽  
pp. 2025-2035
Author(s):  
María Sol Ruiz ◽  
María Belén Sánchez ◽  
Yuly Masiel Vera Contreras ◽  
Evangelina Agrielo ◽  
Marta Alonso ◽  
...  
Keyword(s):  
Latin America ◽  
Myeloid Leukemia ◽  
World Health ◽  
Molecular Response ◽  
Limit Of Quantification ◽  
International Scale ◽  
Deep Molecular Response ◽  
Health Organization ◽  
First Time

AbstractObjectivesThe quantitation of BCR-ABL1 mRNA is mandatory for chronic myeloid leukemia (CML) patients, and RT-qPCR is the most extensively used method in testing laboratories worldwide. Nevertheless, substantial variation in RT-qPCR results makes inter-laboratory comparability hard. To facilitate inter-laboratory comparative assessment, an international scale (IS) for BCR-ABL1 was proposed.MethodsThe laboratory-specific conversion factor (CF) to the IS can be derived from the World Health Organization (WHO) genetic reference panel; however, this material is limited to the manufacturers to produce and calibrate secondary reference reagents. Therefore, we developed secondary reference calibrators, as lyophilized cellular material, aligned to the IS. Our purpose was both to re-evaluate the CF in 18 previously harmonized laboratories and to propagate the IS to new laboratories.ResultsOur field trial including 30 laboratories across Latin America showed that, after correction of raw BCR-ABL1/ABL1 ratios using CF, the relative mean bias was significantly reduced. We also performed a follow-up of participating laboratories by annually revalidating the process; our results support the need for continuous revalidation of CFs. All participating laboratories also received a calibrator to determine the limit of quantification (LOQ); 90% of them could reproducibly detect BCR-ABL1, indicating that these laboratories can report a consistent deep molecular response. In addition, aiming to investigate the variability of BCR-ABL1 measurements across different RNA inputs, we calculated PCR efficiency for each individual assay by using different amounts of RNA.ConclusionsIn conclusion, for the first time in Latin America, we have successfully organized a harmonization platform for BCR-ABL1 measurement that could be of immediate clinical benefit for monitoring the molecular response of patients in low-resource regions.

scholarly journals Neonatal screening and genotype-phenotype correlation of hyperphenylalaninemia in the Chinese population

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Xin Wang ◽  
Yanyun Wang ◽  
Dingyuan Ma ◽  
Zhilei Zhang ◽  
Yahong Li ◽  
...  
Keyword(s):  
Neonatal Screening ◽  
Phenylalanine Hydroxylase ◽  
Laboratory Data ◽  
Molecular Characteristics ◽  
Phenotype Correlation ◽  
Common Amino Acid ◽  
Appropriate Treatment ◽  
Clinical Presentations ◽  
Average Current

Abstract Background Hyperphenylalaninemia (HPA) is the most common amino acid metabolic disease involving phenylalanine hydroxylase (PAH, OMIM*612,349) deficiency or coenzyme tetrahydrobiopterin (BH4) deficiency. Patients with severe HPA often have a difficult life. Early diagnosis of HPA before the development of symptoms is possible via neonatal screening, facilitating appropriate treatment and reducing mortality and disability rates. This study revealed the prevalence, mutational and phenotypic spectrum, and prognosis of HPA by neonatal screening from January 2001 to September 2020 in Nanjing, Jiangsu Province, China. Methods Through a retrospective analysis of the information available in the neonatal screening database, the clinical presentations, laboratory data, molecular characteristics and treatment follow-up data of HPA patients detected by neonatal screening were evaluated. Results We diagnosed 181 patients with HPA from 1 to 957 newborns, giving an incidence of 1:6873. Among these patients, 177 were identified as PAH deficient and four patients were BH4 deficient. The average current age of the patients was 6.38 years old. The most common mutations of PAH were c.728 C > A/ p.Arg243Gln (13.83 %), c.158G > A/ p.Arg53His (9.57 %), c.611 A > G/ p.Tyr204Cys (7.44 %), and c.721 C > T/ p.Arg241Cys (6.38 %). Conclusions This study revealed the prevalence, phenotype-genotype, and prognosis of HPA in China and contributes to the updating of PAHD data for China and worldwide. Our study not only expanded the spectrum of phenotypes and genotype but also provided a valuable tool for improved genetic counseling and management of future cases.

scholarly journals Detection of Rickettsia lusitaniae Among Ornithodoros sawaii Soft Ticks Collected From Japanese Murrelet Seabird Nest Material From Gugul Island, Republic of Korea

2021 ◽  
Author(s):  
Heung-Chul Kim ◽  
Ju Jiang ◽  
Jun Hang ◽  
Su Yeon Kim ◽  
Seok-Min Yun ◽  
...  
Keyword(s):  
Coastal Area ◽  
Nested Pcr ◽  
Republic Of Korea ◽  
Nest Material ◽  
Antigen Gene ◽  
The Republic ◽  
First Time

Abstract In a follow-up to the investigations of soft ticks identified from seabird nest soil and litter collected from coastal islands of the Republic of Korea (ROK), Ornithodoros sawaii and Ornithodoros capensis were assessed for the presence and identification of rickettsiae. Ticks collected from samples of 50–100 g of nest litter and soil from seabird nests were identified individually by morphological techniques, and species confirmed by sequencing of the mt-rrs gene. Subsequently, tick DNA preparations were screened for the presence of rickettsiae using a genus-specific nested PCR (nPCR) assay targeting the 17 kDa antigen gene. The amplicons from the 17 kDa assay and two additional nPCR assays targeting the gltA and ompB gene fragments were sequenced and used to identify the rickettsiae. A total of 134 soft ticks belonging to two species, O. sawaii Kitaoka & Suzuki 1973 (n = 125) and O. capensis Neumann 1901 (n = 9), were collected. Rickettsia lusitaniae DNA was detected and identified among O. sawaii ticks (n = 11, 8.8%) collected from nest litter and soil of the Japanese murrelet (Synthliboramphus wumizusume Temminck 1836) at Gugul Island along the western coastal area of the ROK. This study confirmed for the first time the presence of R. lusitaniae associated with O. sawaii collected from migratory seabird nests in the ROK.

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