A comparative analysis of clinical characteristics of patients with paroxysmal nocturnal hemoglobinuria between Asia and Europe/America

2016 ◽  
Vol 103 (6) ◽  
pp. 649-654 ◽  
Author(s):  
Fan Yu ◽  
Yali Du ◽  
Bing Han
2021 ◽  
Author(s):  
Kayo Nakata ◽  
Richard Williams ◽  
Yoshiaki Kinoshita ◽  
Tsugumichi Koshinaga ◽  
Veronica Moroz ◽  
...  

2021 ◽  
Vol 10 (4) ◽  
pp. 744
Author(s):  
Herman Yosef Limpat Wihastyoko ◽  
Arviansyah Arviansyah ◽  
Erdo Puncak Sidarta

Work from home (WFH) mandate is one of the major changes known during this pandemic, aimed as a preventive way to mitigate the spread of the COVID-19 virus. This study aimed to observe the characteristics of pediatric burn injury during COVID-19 pandemic and WFH mandate's impact on pediatric burn injury admission at some Hospital burn centers in Malang. Every patient’s age, gender, clinical characteristics, parent's background, and other variables such as the possession of siblings, response time using our burn registry form, and comparative analysis of the incident in WFH housewife mother were assessed. The majority were in the group age of under five years old group age (70%) with a mean of 5.5 years. The most frequent part of the burn injured is extremity 36.7%, and hot liquid dominates as the cause of the injury 73.3% with the total body surface area of burn injury group >10% is the most common 56.7%. The burn injury incident happened more frequently in mothers with children less than two in both groups. This study showed that the increase in increasement of the pediatric burn injury during COVID-19 pandemic between housewife mother and WFH mother has no significant difference also showed that parent especially mother unable to supervise the children during WFH. Strategies to mitigate pediatric burn injuries during WFH should be thoughtfully implemented.


2017 ◽  
Vol 33 (8) ◽  
pp. 405-410 ◽  
Author(s):  
Hui-Ching Wang ◽  
Ching-Yuan Kuo ◽  
I-Ting Liu ◽  
Tsai-Yun Chen ◽  
Yu-Hsiang Chang ◽  
...  

2020 ◽  
Vol 99 (7) ◽  
pp. 1505-1514 ◽  
Author(s):  
Hubert Schrezenmeier ◽  
Alexander Röth ◽  
David J. Araten ◽  
Yuzuru Kanakura ◽  
Loree Larratt ◽  
...  

2007 ◽  
Vol 0 (0) ◽  
pp. 071121035321001-??? ◽  
Author(s):  
Soon Thye Lim ◽  
Siew Wan Hee ◽  
Richard Quek ◽  
Lay Cheng Lim ◽  
Swee Peng Yap ◽  
...  

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1752.1-1752
Author(s):  
B. Hernández-Cruz ◽  
F. J. Olmo Montes ◽  
M. J. Miranda García ◽  
M. D. Jimenez Moreno ◽  
M. A. Vázquez Gómez ◽  
...  

Background:Fragility fractures (FF) represent a health problem and among them, the VFF. They have worse vital prognosis, are at greater risk of new FF, had higher comorbidity, with clinical manifestations in only 30%-40% of cases. One in 6 women and one in 12 adult males will have a VFF.Objectives:To analyze the clinical characteristics of FF patients attended in the FLS at Virgen Macarena University Hospital. Compare the sociodemographic and clinical characteristics of VFF patients with those with OFF.Methods:Design: Prospective cohort. Patients attended in the FLS from May 2018 to November 2019 in a protocolized manner (Openclinica®). Inclusion criteria: a clinical FF in the previous two years. Descriptive statistics: percentages and means with 25thand 75thpercentile. Inferential statistic by parametric and nonparametric tests. The project was approved by the Ethics Committee and patients signed consent to participate.Results:Data from 414 patients with a first FF are analyzed, 101 (25%) with VFF and 313 (76%) with OFF [188 (45%) hip, 66 (16%) distal radius, 32 (8%) humerus and 27 (6%) miscellaneous (pelvis, ribs, tibia)]. All VFFs analyzed had clinical symptoms and the number of fractured vertebrae was 2 (1-3). In 28 (37%) were FF of dorsal vertebrae, at 25 (33%) lumbar and 23 (30%) dorsal and lumbar. Comparative analysis showed differences in age VFF 71 (62-77) vs OFF 76 (66 – 83) years, p=0.0003. It highlighted a bimodal distribution according to age, with a peak incidence of 55 to 68 years and another between 75-80 years (Graph). Referral unit to FLS: VFF Rheumatology (42%) and/or Traumatology Emergency Room (44%) vs OFF Internal Medicine (45%) and General Traumatology Unit (38%), p=0.0001. There were also differences in the treatment with teriparatide (VFF 20% vs OFF 4%); zoledronate (VFF 6% vs OFF 3%) and alendronate (VFF 44% vs OFF 63%, p=0001); days of immobilization (VFF 30 (0 - 60) vs OFF 10 (0 - 30), p-0.01); they have greater independence to carry out activities of daily life (Barthel Scale) VFF 95(81 – 100) vs OFF 80 (60 – 95), p=0.00001; increased clamping force of hands 18 (12 - 20) vs 12 (8 - 18) mmHg, p=0.001, and lower risk of falls (J D Downton Scale) (VFF 43% vs OFF 60%, p=0,01). While the number of relevant comorbidities was higher in VFF 3 (1 - 5) vs OFF 2 (1 - 4) it was no statistical, p=0.3. The use of GCC was risky for VFF (n=13, 13%) vs OFF (n=17, 5%), p=0.01 and RR (95%CI) 2.3 (1.01 – 5.3) and not for other drugs (GnRH inhibitors, aromatase inhibitors or chemotherapy). No differences in sex were found (VFF 80%- vs OFF 80% women, p=0.9), previous FF history (9% vs 12%, p=0.2), secondary OP (16% vs 21%, p=0.1); percentage of patients with OP by femoral neck DEXA (VFF 35% vs 42%, p=0.2) or by lumbar spine DEXA (VFF 36% vs OFF 34%, p=0.8).Conclusion:VFF have a bimodal age-based distribution, usually occurring in younger patients, with a higher degree of independence and muscle strength and lower risk of falls, although they are associated with longer duration of immobilization, compared to OFF. In our cohort, VFFs affect 2 or more vertebrae and they are commonly treated with parenteral osteoporotic drugs. The use of glucocorticoids doubled the risk of developing a VFF, these findings are similar to those of others published cohorts.This project received a grant of the Ministry Health of the Junta de Andalucía Ref.PIN-0092-2016.Bibliography:[1]Gerdhem P. Best Practice & Research Clinical Rheumatology 27 (2013) 743–755[2]McCarty J,et al. Diagnosis and management for vertebral compression fractures. American Family of Physicians Jul1, 2016 Vol 94 No 1.Disclosure of Interests:Blanca Hernández-Cruz Speakers bureau: Abbvie, Lilly, Sanofi, BMS, STADA, Francisco Jesús Olmo Montes: None declared, Maria José Miranda García: None declared, María Dolores Jimenez Moreno: None declared, María Angeles Vázquez Gómez: None declared, Mercedes Giner García: None declared, Miguel Angel Colmenero Camacho: None declared, José Javier Pérez Venegas: None declared, María José Montoya García: None declared


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 3341-3341
Author(s):  
Alvaro Urbano-Ispizua ◽  
Petra Muus ◽  
Hubert Schrezenmeier ◽  
Antonio Medina Almeida ◽  
Amanda Wilson ◽  
...  

Abstract Introduction: Studies of children with paroxysmal nocturnal hemoglobinuria (PNH) are scarce and include a very limited number of patients. The objective of this analysis was to describe characteristics of PNH at enrollment for the largest available registry of pediatric patients, and to compare demographic and clinical characteristics with those of adult patients. Methods: The International PNH Registry is a prospective, multi-center worldwide, observational study of patients with a PNH clone of 0.01-100%. Data are collected from patient medical records at the time of Registry enrollment and every six months thereafter. Adult patients were ³18 years of age at enrollment and disease start and pediatric patients were <18 years at enrollment. Demographics and clinical parameters in patients untreated with eculizumab at enrollment for the two age cohorts were compared using the Wilcoxon-Mann-Whitney test for medians and PearsonÕs chi-square for frequencies. The rate of thrombotic events (TE) between disease start (defined as the earliest reported PNH symptoms, granulocyte clone, or PNH diagnosis) and enrollment was calculated per 100 person-years. Results: As of March 2, 2015, a total of 2,184 patients were eligible for analysis: 94 (4.3%) pediatric patients and 2,090 (95.7%) adult patients. Median age (range) at enrollment was 14.0 years (3-17) in pediatrics and 45.5 years (18-100) in adults; median disease duration was 0.7 years and 2.1 years, respectively (p<0.001). More pediatric than adult patients had a PNH clone of <10% and severe cytopenia (Table). Pediatric patients had lower percent of reticulocytes compared with adults (2.1% vs. 2.6%, respectively; p=0.015). History of aplastic or hypoplastic anemia was more frequent in pediatric than adult patients (76.5% vs 54.4%, respectively; p<0.001). History of TE and any major adverse vascular event was less frequent in pediatrics (2.1% vs 8.7%; p=0.025, and 4.3% vs. 14.4%; p=0.005). The rate of TE between disease start and enrollment was lower in pediatric patients, but not statistically significant: 1.4 per 100 person-years (95%CI 0.2-5.2) compared to adult patients (2.3 per 100 person-years (95%CI 2.0-2.6). More pediatric patients than adults had abdominal pain at enrollment. Conclusions: The International PNH Registry provides the largest available pediatric cohort of patients with a PNH clone to characterize this understudied population and demonstrate an important disease burden. Pediatric patients were more likely to have smaller PNH clones and a higher component of aplastic/hypoplastic anemia. Pediatric patients had fewer vascular events. These findings may reflect the natural evolution of the disease and can be useful in the clinical management of PNH. Table 1. Clinical Characteristics at Enrollment of Pediatric and Adult Patients with PNH Pediatric(n=94) Adult(n=2,090) P-value Clone size (percent GPI-deficient granulocytes), n (%)<10% 10 to < 50% ³50% 47 (55.3) 16 (18.8) 22 (25.9) 550 (38.3) 322 (22.4) 565 (39.3) 0.006* Cytopenia status, n (%)None (neutrophils ³ 1.5 x 109/L and platelets ³100 x 109/L) Moderate (neutrophils <1.5 x 109/L or platelets <100 x 109/L) Severe (neutrophils <0.5 x 109/L or platelets <20x109/L) 22 (29.3) 28 (37.3) 25 (33.3) 735 (42.2) 784 (45.1) 221 (12.7) <0.001* Percent reticulocytesMedian (Q1, Q3) 2.1 (1.1, 3.5) 2.6 (1.6, 4.6) 0.015 Hemolytic status, n (%)Hemolytic (LDH ³1.5 x ULN and/or reticulocytes ³60 x 109/L) Not hemolytic (LDH <1.5 x ULN and reticulocytes <60 x 109/L) 33 (58.9) 23 (41.1) 1,038 (65.3) 551 (34.7) NS LDH Ratio, n (%)<1.5 x ULN³1.5 x ULN 30 (58.8) 21 (41.2) 684 (47.0) 770 (53.0) NS History of TE, n (%)Yes No 2 (2.1) 92 (97.9) 181 (8.7) 1,902 (91.3) 0.025 Rate of TENumber of TE, n Person-years (disease start to enrollment) Rate/100 person-years (95% CI) 2 139.9 1.4 (0.2-5.2) 255 11,119.8 2.3 (2.0-2.6) NS History of MAVE, n (%) 4 (4.3) 300 (14.4) 0.005 GPI, glycosylphosphatidylinositol; LDH, lactate dehydrogenase; MAVE, major adverse vascular event; TE, thrombotic event; ULN, upper limit of normal *P-values for clone size and cytopenia status represent overall comparison of categories. Disclosures Muus: Alexion Pharmaceuticals: Honoraria. Schrezenmeier:Alexion Pharmaceuticals: Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau. Almeida:Celgene: Consultancy; Novartis: Consultancy; Bristol Meyer Squibb: Speakers Bureau; Shire: Speakers Bureau. Wilson:Alexion Pharmaceuticals: Employment. Ware:Bayer Pharmaceuticals: Consultancy; Biomedomics: Research Funding; Eli Lilly: Other: DSMB membership; Bristol Myers Squibb: Research Funding.


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