Vogt-Koyanagi-Harada disease in Spain

2021 ◽  
pp. 112067212110334
Author(s):  
Pedro Arriola-Villalobos ◽  
Aina Moll-Udina ◽  
Mario Carrasco-López-Brea ◽  
Cristina Sacristan ◽  
Maria Jose Capella ◽  
...  

Purpose: To describe the clinical and epidemiological characteristics of patients with Vogt-Koyanagi-Harada (VKH) disease in Spain. Methods: This was a retrospective multicenter analysis of data from VKH patients followed for at least 6 months. The data collected were related to demographics, clinical manifestations, treatments, and complications. Results: Participants were 112 patients (224 eyes), from 13 tertiary referral centers, of mean age 37.5 ± 14.7 years; 83.9% were women. Ethnicities were 61.6% Caucasian and 30.4% Hispanic. The disease was classified as complete in 16.1%, incomplete in 55.4%, and probable in 28.6%. When seen for the first time, the clinical course was acute in 69.6%, recurrent chronic in 15.2%, and chronic in 14.3%. The most frequent treatment was corticosteroids (acute stage 42.2%, maintenance stage 55.6%). The most common complications were cataract (41.1%) and ocular hypertension (16.1%). In most eyes, visual acuity was improved (96.7%) or remained stable at the end of follow up. Conclusion: VKH in Spain mostly affects women and presents as incomplete acute stage disease. Visual prognosis is good. Cataract and glaucoma are the two most frequent complications.

2020 ◽  
Vol 31 (1) ◽  
Author(s):  
Dayami Martínez Sosa ◽  
Oswaldo Vásconez Hatt ◽  
Katherine Rosero Arboleda ◽  
Fabian Zurita Alvarado ◽  
Mónica Hernández Lojano ◽  
...  

By the ending of 2019 a new type of coronavirus was identified named SARS-CoV 2, and now known to be the etiological agent of the acute respiratory syndrome coronavirus (SARS-CoV-2). Known as coronavirus 2019 disease or (COVID-19) constitutes a challenge for the world in many unknown aspects and problems in public health. In Ecuador the first reported of a confirmed appeared on February 29 2020, and in March 12 an emergency health status is declared. Objective: To determine the epidemiological characteristics and factors related to death and survival in patients with COVID-19. Design: The study is of transversal retrospective design, the patients chosen were those seen in the respiratory or suspicious of COVID-19 area of Hospital Vozandes Quito between March 2 and April 30. Demographic data and related risk factors for mortality were obtained using the EPI-1 individual form, epidemiological research files, the VI Epi system and electronic promptuary of patients. Results: 250 patients were obtained for this study, 87 of those were confirmed COVID-19, out those eight died and 79 were alive at the ending of the follow up. Regarding the severity of illness, 61% of the patients were classified as mild and 6 % critical (p =<0.001). Elevated values of CRP (OR 1 IC 95% 1.000 – 1.024), age about 55 years old to diagnosis (OR 42,040 IC 95% 36,320 – 47,760) and the presence of hiporexia (OR 24 IC 95% 1.183 – 504.413) were associated with higher mortality levels amongst COVID-19 positive patients. Conclusions The majority of COVID-19 cases showed no serious manifestations, were treated symptomatically and home isolation. Elevated values of CRP and the presence of hiporexia at the diagnosis are factors consistently with death. Future investigations are required to determine the risk factors associated with worst clinical course and prognosis.


2020 ◽  
Author(s):  
Lu Zhang ◽  
Lan Dong ◽  
Lei Ming ◽  
Min Wei ◽  
Jun Li ◽  
...  

Abstract Background Compared with Severe Acute Respiratory Syndrome(SARS) and Middle East Respiratory Syndrome(MESR), Corona Virus Disease 2019(COVID-19) spread more rapidly and widely. The population was generally susceptible. However, reports on pregnant women infected with SARS-CoV-2 were very limited. By sharing the clinical characteristics, treatments and outcomes of 18 patients with COVID-19 during late pregnancy, we hoped to provide some references for obstetric treatment and management. Methods: A total of 18 patients with COVID-19 treated in Renmin Hospital of Wuhan University were collected. The epidemiological characteristics, clinical manifestations, laboratory tests, chest CT and pregnancy outcomes were performed for analysis. Results: 1.18 cases of late pregnancy infected with SARS-CoV-2 pneumonia were delivered at 35 + 5 weeks to 41 weeks. According to the clinical classification of COVID-19, 1 case was mild type, 16 cases were ordinary type, and 1 case was severe type. 2 . According to Imaging examinations: 15 (83%) cases showed unilateral or bilateral pneumonia, 2 (11%) cases had pulmonary infection with pleural effusion, and 1(6%) case had no abnormal imaging changes. 3. Among the 18 newborns, there were 3 (17%) premature infants, 1 (6%) case of mild asphyxia, 5 (28%) cases of bacterial pneumonia, 1 (6%) case of gastrointestinal bleeding, 1 (6%) case of necrotizing enteritis, 2 (11%) cases of hyperbilirubinemia and 1 (6%) case of diarrhea. All the newborns were negative for the first throat swab test of SARS-CoV-2 after birth. 4. Follow-up to Mar 7, 2020, no maternal and neonatal deaths occurred. Conclusions: The majority of patients in late term pregnancy with COVID-19 were of ordinary type, and they less likely developed into critical pneumonia after early isolation and antiviral treatment. Vertical transmission of SARS-CoV-2 was not detected, but the proportion of neonatal bacterial pneumonia was higher than other neonatal diseases in newborns.


Author(s):  
Qin Wu ◽  
Yuhan Xing ◽  
Lei Shi ◽  
Wenjie Li ◽  
Yang Gao ◽  
...  

BackgroundSevere acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a newly identified pathogen which mainly spreads by droplets. Most published studies focused on adult patients with coronavirus disease 2019 (COVID-19), but data concerning pediatric patients is limited. This study aimed to determine epidemiological characteristics and clinical features of pediatric patients with COVID-19.MethodsWe reviewed and analyzed data on pediatric patients with laboratory-confirmed COVID-19, including basic information, epidemiological history, clinical manifestations, laboratory and radiologic findings, treatment, outcome and follow-up results.ResultsFrom January 20th to February 27th of 2020, a total of 74 pediatric patients with COVID-19 were included in this study. Of the 68 cases whose epidemiological data were complete, 65 (65/68, 95.59%) cases were household contacts of adults whose symptoms developed earlier. Forty (59.46%) of the infected children were male, and the median age and body weight are 6 (0.10-15.08) years and 24 (4.20-87.00) kg, respectively. Except for one critically ill case, 20 (27.03%) patients did not show any symptoms of infection, 24 (32.43%) patients had acute upper respiratory tract infection and 29 (39.19%) patients were diagnosed with mild pneumonia. Cough (24/74, 32.43%) and fever (20, 27.03%) were the predominant symptoms of 44 (59.46%) symptomatic patients at onset of the illness. Abnormalities in leukocyte count were found in 23 (31.08%) children and 10 (13.51%) children presented with abnormal lymphocyte count. Of the 34 (45.95%) patients who had nucleic acid testing results for common respiratory pathogens, 19 (19 / 34, 51.35%) showed co-infection with other pathogens other than SARS-CoV-2.Ten (13.51%) children had RT-PCR analysis of SARS-CoV-2 for fecal specimens and 8 of them showed prolonged existence of SARS-CoV-2 RNA 11 (5-23) days after nasopharynx swabs turning negative. Abnormalities in chest imaging were observed in 37 (50.00%) patients and 28 (37.84%) of them only presented with nontypical changes.All children had good prognosis with a median hospitalization duration of 11 days and follow-up period of 16.5 days. During the follow-up period, all the patients remained in quarantine at designated site and home for two 14-day obervation periods and showed no clinical manifestation,but 3 of the 8 cases with prolonged fecal shedding of SARS-CoV-2 still showed positive result of feces test.


2003 ◽  
Vol 13 (5) ◽  
pp. 491-495
Author(s):  
M. Rinaldi ◽  
A. Villani ◽  
M. Borrelli ◽  
S. Russo ◽  
L. Cotticelli

Purpose To describe the clinical manifestations in a patient with Sjögren reticular dystrophy of the retinal pigment epithelium, and the evolution of the disease over a 20-year follow-up period. Case Report A 45-year-old woman with Sjögren reticular dystrophy of the retinal pigment epithelium was seen for the first time in 1983; the patient underwent 20 years of annual check-ups. Results Over the follow-up period, fundus photographs, computerized perimetry, electroretinogram, and electro-oculogram findings had either normal or slightly subnormal outcome. Conclusions The results confirm that this disease involves only the retinal pigment epithelium and should not be considered a central tapeto-retinal degeneration or late onset retinopathy.


Author(s):  
Raul Alfaro Rangel ◽  
Sabine Lepper ◽  
Nóra Szentmáry ◽  
Achim Langenbucher ◽  
Berthold Seitz

Abstract Purpose To assess prevalence, clinical manifestations, required keratoplasties, follow-up, and outcome in patients with Herpes Simplex Virus Keratitis (HSK) attending a University Tertiary Referral Center. Design Retrospective (12 years), descriptive, observational study. Methods A total of 817 eyes with clinical diagnosis of HSK from 779 patients were classified by the type of presentation. We gathered data on the visual acuity, refraction, IOP, and required surgical procedures. Results Stromal involvement including scars represented the most common diagnosis in our department and the main indication of penetrating keratoplasty (PKP). Epithelial keratitis (16%) presented with the best visual acuity at the first visit. Necrotizing keratitis represented 17% of the patients, 78% of whom required PKP; this group also had the worst visual acuity at first examination and was the main indication for emergency PKP. Among all eyes, 288 (35%) required PKP. A total of 230 (28%) PKPs were elective procedures and 58 (7%) PKPs were performed as emergency procedures. Two patients with quiet endothelial decompensations after recurrent HSV endotheliitis were treated with DMEK and had good visual outcomes without HSV recurrence at last follow-up. Conclusions HSK is a prevalent disease with severe consequences when not treated appropriately and on time. Even when making an accurate diagnosis, the disease can be extremely aggressive, with all the implications it brings to the patients and health system. Elective PKP had better outcomes in terms of visual acuity and clear graft percentage compared to emergency PKP.


2008 ◽  
Vol 14 (8) ◽  
pp. 1139-1141 ◽  
Author(s):  
M Arias ◽  
XR Osorio ◽  
D Dapena ◽  
S Arias-Rivas ◽  
F Vázquez

Diagnosis of primary angiitis of the central nervous system (PACNS) is difficult in relation to variability in its clinical manifestations and absence of specific signs in neuroimaging. A young patient presented with a recurrent encephalopathic clinical course. T2 and fluid-attenuated inversion recovery-magnetic resonance imaging (FLAIR-MRI) showed hyperintense lesions in the cerebral white matter suggesting demyelination. Those lesions decreased or even disappeared after treatment with steroids and immunoglobulins. In echo gradient MRI (T2*-MRI), there were permanent cortical-subcortical petechial hypointense lesions (microhemorrhages). Definite diagnosis was established after cerebral biopsy. Intravenous cyclophosphamide was administrated with no new relapses in more than 18 months of follow-up. In a compatible clinical course, the finding of petechial hemorrhages in T2*-WI could play an important role in early diagnosis of PACNS.


Children ◽  
2021 ◽  
Vol 8 (7) ◽  
pp. 613
Author(s):  
Michele Ghezzi ◽  
Enza D’Auria ◽  
Andrea Farolfi ◽  
Valeria Calcaterra ◽  
Alessandra Zenga ◽  
...  

Background: Few studies have been carried out with the aim of describing the clinical course and follow-up of patients with tracheomalacia. We aim to describe the symptoms at diagnosis and the post-treatment clinical course of patients affected by airway malacia. Methods: We retrospectively analyzed characteristics of pediatric patients with a diagnosis of airway malacia. Patients were classified into three groups: bronchomalacia (BM), tracheomalacia (TM) and tracheo-bronchomalacia (TBM). Demographic and clinical data, diagnostic work-up and surgical treatment were recorded. Results: 13/42 patients were affected by congenital syndromes (30.9%). Esophageal atresia with or without tracheal-esophageal fistula (EA/TEF) was detected in 7/42 patients (16.7%). Cardiovascular anomalies were found in 9/42 (21.4%) and idiopathic forms in 13/42 (30.9%). BM occurred in 7/42 (16.6%), TM in 23/42 (54.7%) and TBM in 12/42 (28.6%). At the diagnosis stage, a chronic cough was reported in 50% of cases with a higher prevalence in EA/TEF (p = 0.005). Surgery was performed in 16/42 (40%) of children. A chronic cough and acute respiratory failure were correlated to the need for surgery. During follow-up, there was no difference in persistence of symptoms between conservative vs surgical treatment (p = 0.47). Conclusion: the management of tracheomalacia remains a challenge for pediatricians. Clinical manifestations, such as a barking cough and acute respiratory failure may suggest the need for surgery. Follow-up is crucial, especially in those patients affected by comorbidities, so as to be able to manage effectively the possible persistence of symptoms, including those that may continue after surgical treatment.


Hematology ◽  
2006 ◽  
Vol 2006 (1) ◽  
pp. 205-210 ◽  
Author(s):  
Charles A. Schiffer

Abstract The management of patients with myelodysplasia (MDS) can be quite complex and varies according to both the clinical manifestations in individual patients as well as complicating medical conditions. Allogeneic stem cell transplantation is the only curative treatment, but because of the older age of the patient population must be applied selectively, particularly in those with lower risk MDS as well as in patients whose clinical course is more frankly “preleukemic.” Issues pertinent to the use of 5-azacytidine, decitabine and lenalidomide in patients with both higher and lower International Prognostic Staging System (IPSS) stage disease are discussed.


2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 8086-8086
Author(s):  
R. R. Harting ◽  
P. Venugopal ◽  
W. Hsu ◽  
R. Catchatourian ◽  
O. Ogundipe

8086 Background: Patients with CLL have a highly variable clinical course. Genomic aberrations detected by FISH have been shown to correlate with survival and treatment free interval (TFI). Patients with the presence of 17p or 11q deletions (del) either alone or in combination with other cytogenetic abnormalities have the worst prognosis while patients with 13q del as a sole abnormality have the best prognosis. Our objective was to further investigate poor prognosis CLL patients with either del 17p or 11q to determine if the addition of the favorable 13q del influences the predicted clinical course and survival. Methods: We performed a retrospective chart review on 22 patients (pts) who had been identified by FISH as having either the combination of del 17p and 13q or del 11q and 13q. Results: 128 CLL FISH panels were performed from April of 2003 through October of 2006. Twenty-two pts (17%) had either del 17p and 13q (9%) or del 11q and 13q (9%). Historical data notes a frequency of 7% and 8% for deletions 17p and 11q, respectively, and 55% for 13q as a sole aberration. The median age was 66 yrs, the majority of whom were male (73%). Two of 22 pts (9%) presented with advanced stage disease. Splenomegaly was seen more often in the 17p/13q pts (36%) vs 11q/13q pts (9%). With a median follow up of 46 months since diagnosis, the median TFI for all patients (20 known) was 56 months. TFI was 13 months for patients with 17p/13q del; whereas TFI was not reached for patients with 11q/13q. Historical data noted a TFI for patients with deletions 17p, 11q, and 13q (as a sole aberration) as 9, 13, and 92 months respectively. The median survival from diagnosis was not reached for the group overall or for either combination of genetic abnormalities. Historical data noted, with 70 months follow up, a median survival of 108 months overall and 32, 79, and 133 months for 17p, 11q, and 13q (as a sole aberration) respectively. Conclusions: The addition of favorable cytogenetics, del 13q, in a CLL patient with an unfavorable cytogenetic pattern (either del 17p or del 11q) appears to improve the predicted clinical outcome and survival. Additional follow up and prospective studies are needed to further define which genetic subgroups help to prognosticate CLL patients. No significant financial relationships to disclose.


2020 ◽  
Vol 9 (10) ◽  
pp. 3335
Author(s):  
Giulio Tessarin ◽  
Stefano Rossi ◽  
Manuela Baronio ◽  
Luisa Gazzurelli ◽  
Michael Colpani ◽  
...  

Activated phosphoinositide 3-kinase delta syndrome 1 (APDS-1) is a recently described inborn error of immunity caused by monoallelic gain-of-function mutations in the PIK3CD gene. We reviewed for the first time medical records and laboratory data of eight Italian APDS-1 patients. Recurrent sinopulmonary infections were the most common clinical feature at onset of disease. Seven patients presented lymphoproliferative disease, at onset or during follow-up, one of which resembled hemophagocytic lymphohistiocytosis (HLH). Genetic analysis of the PIK3CD gene revealed three novel mutations: functional testing confirmed their activating nature. In the remaining patients, the previously reported variants p.E1021K (n = 4) and p.E525A (n = 1) were identified. Six patients were started on immunoglobulin replacement treatment (IgRT). One patient successfully underwent hematopoietic stem cell transplantation (HSCT), with good chimerism and no GVHD at 21 months post-HSCT. APDS-1 is a combined immune deficiency with a wide variety of clinical manifestations and a complex immunological presentation. Besides IgRT, specific therapies targeting the PI3Kδ pathway will most likely become a valid aid for the amelioration of patients’ clinical management and their quality of life.


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