scholarly journals Hemophagocytic Lymphohistiocytosis Is Not Only a Childhood Disease: A Multi-Center Study of 613 Cases from Chinese HLH Workgroup

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4146-4146 ◽  
Author(s):  
Zhao Wang ◽  
Yini Wang ◽  
Wenqiu Huang ◽  
Liangding Hu ◽  
Xinan Cen ◽  
...  
Keyword(s):  
Poor Prognosis ◽  
Hepatic Function ◽  
Underlying Disease ◽  
The Other ◽  
Hematopoietic Stem ◽  
Childhood Disease ◽  
Ebv Infection ◽  
The Mean ◽  
Multi Center Study ◽  
Center Study

Abstract Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome. It occurs as a primary or acquired disorder in a condition of chaotic and uncontrolled immune system stimulation. Recent studies showed that HLH occurs not only in children, but also in adult. Here we presented the data gathered from Chinese HLH Workgroup in order to make further understanding about the disease. Methods: We performed a multi-center study involving 613 cases of HLH patients. We analyzed the clinical features, laboratory tests, diagnosis, treatments and clinical outcomes of them. All the date came from 46 hospitals, between Jan 2006 to June 2014. Results: The median age was 29 years old (Range 1 month to 82 years old). Forty-nine cases of them were primary HLH, 489 cases were acquired HLH and the other 75 case had unknown underlying disease. Among the acquired HLH group, 211 cases were malignancy-associated HLH (M-HLH),207 cases were infection- associated HLH (I-HLH), 58 cases were macrophage activation syndrome (MAS), 9 cases were associated with pregnancy, 3 patients developed HLH after autologous hematopoietic stem cell transplantation, and one case was associated with hemolytic anemia. Over ninety percent (93.3%) of the patients had hepatic function damage. We found significant difference in some diagnostic criteria among M-HLH, I-HLH and MAS group. The mean leukocytes was (9.50±8.46)×109/L, and the mean thrombocytes was (100.69±87.61)×109/L , which were significantly higher in MAS group than the other. The brain natriuretic peptide (BNP) was higher in patients with M-HLH (5477.31±6048.08 pg/ml). The aspartate aminotransferase (AST) was and bilirubin were significantly higher in patients with I-HLH. The median survival time of 613 cases is 7 months. Until now, 258 cases of patients were still survived, 297 cases were died, and the other was lost. We observed 45 cases who underwent allogenic hematopoietic stem cell transplantation (allo-HCT), including 22 cases of primary HLH, 12 cases of M-HLH, 8 cases of EBV-HLH and 3cases with unknown underlying disease. In these allo-HCT patients, 26 cases were achieved complete remission (CR), 12 cases died from transplantation related mortality (TRM), and the other died from tumor relapse or refractory EBV infection. The dead exhibited much higher level in bilirubin, lactate dehydrogenase (LDH), creatinine, and urea nitrogen. On the contrary, they had lower leukocytes and thrombocytes counts, and hypoalbuminemia. Thrombocytopenia and hypoalbuminemia play an important role in poor prognosis. Conclusion: HLH is not only a childhood disease but also occurs in adult. It associated with many underlying conditions. Lymphoma and EBV infection were two major causes of acquired HLH. Hepatic function damage is one of the most typical HLH clinical manifestations, and it tends to be more serious for patients with I-HLH. In addition, the blood cells of the patients with MAS may not reduce in the early phase; and the patients with M-HLH are easy to catch cardiac dysfunction. Acquired HLH is still a high mortality disease, and allo-HCT is one of the effective means of treatment for acquired HLH. Thrombocytopenia and hypoalbuminemia play an important role in poor prognosis. Disclosures No relevant conflicts of interest to declare.

scholarly journals The Efficacy of Anti-Arrhythmic Drugs in Children With Idiopathic Frequent Symptomatic or Asymptomatic Premature Ventricular Complexes With or Without Asymptomatic Ventricular Tachycardia: a Retrospective Multi-Center Study

2021 ◽  
Author(s):  
Robin A. Bertels ◽  
Janneke A. E. Kammeraad ◽  
Anna M. Zeelenberg ◽  
Luc H. Filippini ◽  
Ingmar Knobbe ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Beta Blockers ◽  
Control Group ◽  
Premature Ventricular Complexes ◽  
Percentage Points ◽  
Asymptomatic Children ◽  
Group A ◽  
The Mean ◽  
Multi Center Study ◽  
Center Study

AbstractThe aim of the study is to compare the efficacy of flecainide, beta-blockers, sotalol, and verapamil in children with frequent PVCs, with or without asymptomatic VT. Frequent premature ventricular complexes (PVCs) and asymptomatic ventricular tachycardia (VT) in children with structurally normal hearts require anti-arrhythmic drug (AAD) therapy depending on the severity of symptoms or ventricular dysfunction; however, data on efficacy in children are scarce. Both symptomatic and asymptomatic children (≥ 1 year and < 18 years of age) with a PVC burden of 5% or more, with or without asymptomatic runs of VT, who had consecutive Holter recordings, were included in this retrospective multi-center study. The groups of patients receiving AAD therapy were compared to an untreated control group. A medication episode was defined as a timeframe in which the highest dosage at a fixed level of a single drug was used in a patient. A total of 35 children and 46 medication episodes were included, with an overall change in PVC burden on Holter of -4.4 percentage points, compared to -4.2 in the control group of 14 patients. The mean reduction in PVC burden was only significant in patients receiving flecainide (− 13.8 percentage points; N = 10; p = 0.032), compared to the control group and other groups receiving beta-blockers (− 1.7 percentage points; N = 18), sotalol (+ 1.0 percentage points; N = 7), or verapamil (− 3.9 percentage points; N = 11). The efficacy of anti-arrhythmic drug therapy on frequent PVCs or asymptomatic VTs in children is very limited. Only flecainide appears to be effective in lowering the PVC burden.

scholarly journals Acute Megakaryocytic Leukemia with or Without Acquired Trisomy 21 in 15 Pediatric Patients

2020 ◽  
Author(s):  
Wenzhi ZHANG ◽  
Hui LI ◽  
Jingzhen LIU ◽  
Jiawei XU ◽  
Jinjin HAO ◽  
...  
Keyword(s):  
Trisomy 21 ◽  
Poor Prognosis ◽  
Clinical Characteristics ◽  
Pediatric Patients ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
The Other ◽  
Hematopoietic Stem ◽  
Acute Megakaryocytic Leukemia

Abstract The knowledge of clinical characteristics and prognosis of pediatric acute megakaryocytic leukemia (AMKL) with or without acquired +21 was limited. We reported 15 AMKL pediatric patients without Down Syndrome (four cases with acquired +21 and 11 cases without acquired +21) with the clinical manifestations, laboratory data, and prognosis. The clinical features and laboratory data between patients with acquired +21 and patients without acquired +21 are similar. As for prognosis, three of the 11 cases without acquired +21 obtained complete remission (CR) after 1st induction. The median follow-up time of the 11 cases was 9 months. Among four cases with acquired +21, one case gave up treatment during 1st induction, one obtained CR after 1st induction and was still alive after 49 months of follow-up. One case obtained CR after 2nd induction and was still alive for 15 months of follow-up after bone marrow transplantation, the other patient was planning for allogeneic hematopoietic stem cell transplantation (HSCT) without CR. The median follow-up time of the four cases was 12 months. None relapsed in our study. In conclusion, acquired trisomy 21 may not be an indicator for poor prognosis. Cytogenetics analysis can help us for diagnosis stratification, prognostic judgment and individualized treatment of AMKL.

scholarly journals Evaluation of Efficacy and Tolerability of Perindopril 8 mg in Bangladeshi Hypertensive Patients: Results from an Open-label, Observational, Multi-center Study (CONTROL)

2012 ◽  
Vol 5 (1) ◽  
pp. 44-50
Author(s):  
MN Islam ◽  
AAS Majumder ◽  
RS Mahmud ◽  
M Sadequzzaman ◽  
MA Bashar ◽  
...  
Keyword(s):  
Stage Ii ◽  
Uncontrolled Hypertension ◽  
Newly Diagnosed ◽  
Open Label ◽  
Hypertensive Patients ◽  
Group I ◽  
The Mean ◽  
Group Ii ◽  
Multi Center Study ◽  
Center Study

Background: The CONTROL (COversyl in Newly diagnosed stage-II & unconTROlled hypertensivepatients triaL) was performed with an objective to evaluate the blood pressure (BP) lowering efficacyand tolerability of Perindopril 8mg in newly diagnosed stage II patients and uncontrolledhypertensive patients among Bangladeshi population.Methods: This was an open-label, observational, multi-center study conducted in consultationcenters for out-patients located in different cities of Bangladesh. Adults, aged above 18 years withnewly diagnosed stage-II hypertension or uncontrolled hypertension, were recruited. Patients weretreated with Perindopril 4mg daily for first 1 week, afterwards uptitrated to Perindopril 8 mg dailyand continued treatment for 12 weeks. Patients were followed-up at week-1, week-4, week-8 andweek-12.Results: In total, 245 patients were enrolled. Among them, 88 were newly diagnosed stage-II (Group-I) and 157 were uncontrolled (Group-II) hypertensive patients. Male and female distribution was57% and 43% respectively. Mean age of patients was 54.5 ±11.7 years.After 12 weeks treatment, there was a significant reduction in BP from baseline (p<0.001) in overallpopulation as well as in Group I and in Group II. In overall population, the mean BP reduction was-31/-15 mmHg (from 163.7/96.8 mmHg to 132.4/81.7 mmHg. In Group I, the reduction was -33/-16mmHg (from 166.5/98.2 mmHg to 133.4/82.0 mmHg) and in Group II, -30/-14 mmHg (from 159.3/95.6 mmHg to 129.1/81.6 mmHg).10 patients (4.1%) had to discontinue the treatment due to adverse effects. Dry cough (2%) andhypotension (1.2%) were the main cause of discontinuation. Perindopril 8mg was well tolerated asindicated by the high proportion of physicians (81%) reporting ‘good’ to ‘excellent’ tolerability atweek 12.Conclusion: This study suggests that Perindopril 8mg is effective and safe in the treatment ofhypertension in Bangladeshi patients. DOI: http://dx.doi.org/10.3329/cardio.v5i1.12272 Cardiovasc. j. 2012; 5(1): 44-50

scholarly journals Epidemiological and Clinical Risk Factors Related to Severe COVID-19 in Iran: A multi-center study

2020 ◽  
Author(s):  
Seyed Mohammad Hashemi-Shahri ◽  
Seyed Mohammad Nasiraldin Tabatabaei ◽  
Alireza Ansari-moghaddam ◽  
Mahdi Mohammadi ◽  
Hassan Okati-Aliabad ◽  
...  
Keyword(s):  
Risk Factors ◽  
Substance Abuse ◽  
Disease Severity ◽  
Underlying Disease ◽  
Severe Condition ◽  
Factors Affecting ◽  
Logistic Regression Models ◽  
Factors Associated ◽  
Multi Center Study ◽  
Center Study

Abstract Background Iran was one of the first countries to be affected by COVID-19. Identifying factors associated with severity of COVID-19 is effective in disease management. This study investigated the epidemiological and clinical features and factors associated with severity of COVID-19 in one of the less privileged areas in Iran.Methods In a multi-center study, all patients admitted to hospitals of Zahedan University of Medical Sciences located in southeastern Iran were investigated from February 29 to April 31, 2020. Demographic, epidemiological and clinical data of patients were extracted from medical records. To explore the risk factors associated with severity of COVID-19, bivariate and multivariate logistic regression models were used.Results Among the 413 patients, 55.5% were male and 145 (35.10%) were in a severe condition at admission time. Multivariate analysis showed that the adjusted odds of the disease severity increased in patients with older age (OR 3.51; 95% CI, 2.28-5.40), substance abuse (OR 2.22; 95% CI, 2.05-5.78) and at least one underlying disease (OR 3.45; 95% CI, 1.01-1.32).Conclusions COVID-19 was more severe in older patients, patients with a history of substance abuse, and patients with at least one underlying disease. Understanding the factors affecting the disease severity can help for clinical management of COVID-19, especially in less privileged areas where fewer resources are available.

Markers for Guillain-Barré syndrome with poor prognosis: a multi-center study

2017 ◽  
Vol 22 (4) ◽  
pp. 433-439 ◽  
Author(s):  
Yuko Yamagishi ◽  
Hidekazu Suzuki ◽  
Masahiro Sonoo ◽  
Satoshi Kuwabara ◽  
Takanori Yokota ◽  
...  
Keyword(s):  
Poor Prognosis ◽  
Guillain Barre Syndrome ◽  
Guillain Barré Syndrome ◽  
Multi Center Study ◽  
Guillain Barré ◽  
Center Study

scholarly journals Clinical and Epidemiological Characteristics of Streptococcus suis Infections in Catalonia, Spain

2021 ◽  
Vol 8 ◽  
Author(s):  
Javier Díez de los Ríos ◽  
Esteban Reynaga ◽  
Merce García-Gonzàlez ◽  
Jordi Càmara ◽  
Carmen Ardanuy ◽  
...  
Keyword(s):  
Streptococcus Suis ◽  
Farm Workers ◽  
Zoonotic Pathogen ◽  
Pig Farm ◽  
The Mean ◽  
Pig Meat ◽  
Multi Center Study ◽  
Epidemiological Characteristics ◽  
Center Study

Introduction:Streptococcus suis (S. suis) is a human zoonotic pathogen of occupational origin, with infection acquired through contact with live pigs or pig meat. Pig farming is one of Catalonia's biggest industries and as a result this region of Spain has one of the highest density pig populations per km2. The aim of our study was to describe the infections caused by S. suis occurring in that area over a 9-year period.Materials and Methods: A retrospective, multi-center study was carried out by searching records from 15 hospitals in Catalonia for the period between 2010 and 2019.Results: Over the study period altogether nine cases of S. suis infection were identified in five hospitals, with five of these cases occurring in the 2018–2019 period. The mean age of patients was 48 ± 8.9 years and all of them were males. Five patients (55.6%) worked in pig farms. The most frequent manifestation of infection was meningitis (5 cases; 55.6%) followed by septic arthritis (3 cases; 33.3%). None of the patients died at 30 days; nonetheless, 4 developed hearing loss as a long-term complication.Conclusion: The most commonly identified S. suis infection was meningitis. Over 50% of the episodes occurred in the last 2 years and have affected pig farm workers. Further surveillance is needed in order to know its prevalence.

scholarly journals Epidemiology and Risk Factor of Lung Cancer in Morocco

2019 ◽  
Vol 16 (1) ◽  
pp. 55-59
Author(s):  
Amal Haimer ◽  
Souad Belamalem ◽  
Faouzi Habib ◽  
Abdelmajid Soulaymani ◽  
Abdelrhani Mokhtari ◽  
...  
Keyword(s):  
Lung Cancer ◽  
Poor Prognosis ◽  
The Other ◽  
Maximum Frequency ◽  
Chemotherapy Treatment ◽  
Male Population ◽  
Risk Of Progression ◽  
The Mean ◽  
Epidemiological Characteristics ◽  
Oncology Center

The aim of this study is to describe the epidemiological characteristics of lung cancer in Morocco. This is a descriptive retrospective analysis of lung cancer cases, diagnosed and treated at Al Azhar Oncology Center in Rabat over a period of 11 years, between 2005 and 2015. Among the 615 cases that were diagnosed with lung cancer at Al Azhar Oncology Center, 85.85% were male and 14.15% female, the mean age of men with lung cancer was 59.8 ± 11.2 years while that of women was 58.6 ± 12 years. During the study period, there were 129 deaths from this type of cancer, of which 89.9% were men and 10.08% were women. The maximum frequency of death was observed in patients aged between 40 and 60 years with 54.80%. Of all diagnosed cases, 2.40% were diagnosed with metastatic disease. The majority of patients received chemotherapy treatment with 47%, followed by patients who received radiotherapy with 42.3%. On the other hand, the study shows that there is an association between the risk of progression to death and smoking. Lung cancer is dreaded especially in the male population with a high number of cases and lethality as well as a poor prognosis.

scholarly journals Reference Values and the Z-Score Values of Tricuspid Annular Plane Systolic Excursion in Chinese Children

2022 ◽  
Author(s):  
Danlei Chen ◽  
Jinghui Guo ◽  
Bo Liu ◽  
Chunhua Zheng ◽  
Guimin Huang ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Reference Values ◽  
Mean Value ◽  
Chinese Children ◽  
Specific Reference ◽  
Z Score ◽  
The Mean ◽  
Multi Center Study ◽  
Different Parts ◽  
Center Study

Abstract Objective To establish age-specific and body surface area (BSA)-specific reference values of Tricuspid Annular Plane Systolic Excursion (TAPSE) for children under 15 years old in China. Study design A retrospective study was conducted in Children's Hospital Attached to the Capital Institute of Pediatrics. A total of 702 cases were included in this research to establish reference values of TAPSE. SPSS 25.0 (IBM) was used for data analysis. Lambda-mu-sigma method was used to calculate and construct the age-specific and BSA-specific percentiles and Z-score curves of TAPSE. Results The mean value of TAPSE increased with age and BSA from 0 to 15 years in a nonlinear way and reached the adult level (17mm) until 1 year old. No difference could be observed in genders. Conclusions TAPSE values increased with age and BSA in Chinese children aged between 0-15 years and there was no difference between boys and girls. A multi-center study from different parts of China is supposed to be conducted in the future to reflect the whole spectrum of TAPSE in Chinese children.

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