Aim. In COVID-19 Pandemic, a new hyperinfl ammatory syndrome was reported with clinical features ... more Aim. In COVID-19 Pandemic, a new hyperinfl ammatory syndrome was reported with clinical features of Kawasaki disease, named PIMS-TS. We want to present a single center experience where the patients were diagnosed with Kawasaki-like in PIMS-TS with cardiac affliction. Material and method: The study was observational and retrospective, enrolled 14 patients fulfi lling the criteria of PIMS-TS with the median age of 9 (IQR, 1.6-11), 9 male (64.2 %) and 5 female (35.8 %). Results: ECG revealed tachycardia and ST-T changes in 60% of patients. In evolution, ECG modified in 20% and consisted of long QT in 7% of cases, bradycardia in 7%, 3% transitory sick sinus syndrome and 3% grade I/II degree atrio-ventricular block. Cardiac disfunction was evidenced in 4 patients (28%), with reduced ejection fraction under 50%, mitral insuffi ciency in 6 (42.8%), pericardial fl uid in 8 (57.1%) and perivascular brightness in 8 (57.1%). The cardiac biomarkers: NT-proBNP (increased in 9), cTroponin T (incr...
The experience of a favorable outcome of an elective major orthopedic surgical procedure performe... more The experience of a favorable outcome of an elective major orthopedic surgical procedure performed in a severe case of hemophilia A with high titer inhibitors is presented. Specific for the presented case is the minimal dosing schedule of rFVIIa (7 days’ consumption of 68,960 µg vs. standard recommended dosage of 270,000 µg), encouraging for better expectation of success in life-threatening
ABSTRACT Advances in hemophilia care lead to decrease of patients’ mortality and to a better life... more ABSTRACT Advances in hemophilia care lead to decrease of patients’ mortality and to a better life expectancy. These improvements raise another problem: patients have to face with supplementary difficulties related to the disease and its treatment, which can generate emotional reactions.
SummaryA number of 513 consecutive patients (494-haemophilia A and 19-haemophilia B) from eight h... more SummaryA number of 513 consecutive patients (494-haemophilia A and 19-haemophilia B) from eight haemophilia treatment centers have been investigated with Bethesda assay for the presence of factor VIII or IX inhibitors. The overall prevalence of inhibitors was 15.20%, 18.48% in severe, 5.60% in moderate and 12.24% in mild forms. The prevalence was higher than reported in most of the western countries. The age at start of substitution (p = 0.9775), the frequent switching of factor concentrates (p = 0.8931) were not relevant factors for the development of inhibitors. It is worth to be mentioned the unexpectedly occurrence of inhibitors in prior inhibitor negative (6/72) patients (during surgical interventions) probably due to their previous scarce substitution, occurrence which seems not being connected with the continuous infusion modality of factor VIII administration (p = 0.8341). In controversial situations, in the field of low titer (≤1 BU/ml) inhibitors for a reliable interpretat...
SummaryHaemophilia, a lifelong congenital bleeding disease, is a highly demanding disorder, due t... more SummaryHaemophilia, a lifelong congenital bleeding disease, is a highly demanding disorder, due to the costs of its replacement therapy. In the absence of this pivotal treatment, life expectancy and quality of life are deleteriously affected.As illustration, we present a 14 years long follow- up of a patient with severe haemophilia A, treated sporadically with fresh plasma, cryoprecipitate and factor concentrates, who developed a giant iliopsoas pseudotumor. Since he was an infant, under on demand therapy with fresh frozen plasma, cryoprecipitate and low doses of factor concentrates he presented many spontaneous bleedings, developing multiple disabling arthropathies. At the age of 14 years, an iliopsoas hematoma occurred, which relapsed several times, developing an iliopsoas pseudotumour. After 5 years, sepsis with Klebsiella was diagnosed. A CT scan revealed fistula between the pseudotumor and the gut. Under antibiotics, the evolution of sepsis improved, but over a period of 10 mon...
A new born child, age: one month, sex: masculin, with acute respiratory distress syndrome, perior... more A new born child, age: one month, sex: masculin, with acute respiratory distress syndrome, perioronasal cyanosis and hypoxemia has been diagnosed (radiological and imagical-CT) with congenital lobar emphysema (CLE) at the level of the right superior lobe. One month after, the new born child has been diagnosed with the aforesaid syndrome, and, now two months old, he has been subjected to a surgical intervention consisting in lobectomy at the level of the right superior lobe. His postsurgical evolution has proved satisfactory with pulmonary reexpansion.
A new born child, age: one month, sex: masculin, with acute respiratory distress syndrome, perior... more A new born child, age: one month, sex: masculin, with acute respiratory distress syndrome, perioronasal cyanosis and hypoxemia has been diagnosed (radiological and imagical – CT) with congenital lobar emphysema (CLE ) at the level of the right superior lobe. One month after, the new born child has been diagnosed with the aforesaid syndrome, and, now two months old, he has been subjected to a surgical intervention consisting in lobectomy at the level of the right superior lobe. His postsurgical evolution has proved satisfactory with pulmonary reexpansion.
Background: The aim of the study was to document cardiovascular clinical findings, cardiac imagin... more Background: The aim of the study was to document cardiovascular clinical findings, cardiac imaging, and laboratory markers in children presenting with the novel multisystem inflammatory syndrome associated with coronavirus disease 2019 (COVID-19) infection. Methods: This real-time internet-based survey has been endorsed by the Association for European Paediatric and Congenital Cardiologists Working Groups for Cardiac Imaging and Cardiovascular Intensive Care. Children 0 to 18 years of age admitted to a hospital between February 1 and June 6, 2020, with a diagnosis of an inflammatory syndrome and acute cardiovascular complications were included. Results: A total of 286 children from 55 centers in 17 European countries were included. The median age was 8.4 years (interquartile range, 3.8–12.4 years) and 67% were boys. The most common cardiovascular complications were shock, cardiac arrhythmias, pericardial effusion, and coronary artery dilatation. Reduced left ventricular ejection fra...
Summary Background: Haemophilia is a congenital disorder of coagulation with high economic burden... more Summary Background: Haemophilia is a congenital disorder of coagulation with high economic burden due to its requirement for an expensive, lifelong replacement therapy, with additional costs for the frequent complications and for the severe handicapping consequences. The objective of this cross-sectional study aimed at giving an insight into the health condition of young haemophiliacs in the absence of a regular prophylactic therapy. Methods: It was conducted on a heterogeneous group of 37 children and adolescents (4–24 years of age), with similar on demand therapeutic regimen, coming from the whole country, focusing on the joint status by using the Haemophila Joint Health Score (HJHS) system and on quality of life (QoL) by using the EQ-5D-3L-Y questionnaire. Results: The results revealed an impressive situation: 70.3 % with chronic arthropathy, 19 % with target joints, 69 % with multiple joint involvement, mainly elbow (41 %) and knee (34 %), joint damage starting in the age group ...
Objective In countries with low factor concentrate consumption, disabling joint disease remains t... more Objective In countries with low factor concentrate consumption, disabling joint disease remains the major morbidity in patients with haemophilia. The objective of the present analysis is to express the trend and profile of invasive orthopaedic interventions in our country with low usage of factor replacement, lacking the prophylaxis program until recent years. Patients and Methods This retrospective descriptive study was conducted in our university centre in Timisoara with long-lasting experience in haemophilia care, which succeeded in developing an exceptionally valuable genuine comprehensive inter-institutional cooperation. This study refers to 115 invasive interventions performed on 97 patients: 83 with haemophilia A, 10 with haemophilia B and 4 with von Willebrand disease in the period of 2001 to 2017; 17 of them had inhibitors, 5 developing inhibitors after the intervention. Results and Discussion The cohort of patients consisted mostly of young adults, aged between 7 and 49 ye...
The development of FVIII/FIX inhibitor alloantibodies represents a severe complication requiring ... more The development of FVIII/FIX inhibitor alloantibodies represents a severe complication requiring specific laboratory evaluation for establishing a life-saving therapy regimen. Our preliminary study aimed at elaborating a laboratory strategy for monitoring the effectiveness of Activated Prothrombin Complex Concentrate (APCC) and recombinant activated factor VII (rFVIIa) in haemophiliacs with inhibitors, by checking the reliability and clinical value of three complementary assays: clotting-time based coagulometry, thrombelastography (TEG) and thrombin generation assay (TGA). The investigations were performed on 7 patients with severe haemophilia A with high titer inhibitors treated for 12 episodes of severe bleedings, 5 of them for surgical interventions. After the administration of bypassing agents (BPAs) the clotting-time based assay brought changes only on prothrombin (p<0.01), potentially signaling a thrombotic risk, without any impact on the global hemostasis. TEG displayed pr...
Idiopathic thrombocytopenic purpura (ITP) in children is a heterogeneous disease from the point o... more Idiopathic thrombocytopenic purpura (ITP) in children is a heterogeneous disease from the point of view of its natural course and pattern of treatment response. Even in uncomplicated ITP, there is a large variation in disease presentation and prognosis. Although in children it is usually a self limited disorder [1], chronicity (evolution longer than 6 months) and life-threatening bleeds are the main challenges of patients with ITP, justifying preoccupations for their prediction and prevention [2].
... Mean personal income per month in our patients was: preschool children-10.5 € (SD = 0), stude... more ... Mean personal income per month in our patients was: preschool children-10.5 € (SD = 0), students-11.32 € (SD = 6.34), university students-35 ... Haemophilia 1998; 4:491-497 Szucs TD,Offner A, Schramm W. Socioeconomic impact of haemophilia care: results of a pilot study. ...
Aim. In COVID-19 Pandemic, a new hyperinfl ammatory syndrome was reported with clinical features ... more Aim. In COVID-19 Pandemic, a new hyperinfl ammatory syndrome was reported with clinical features of Kawasaki disease, named PIMS-TS. We want to present a single center experience where the patients were diagnosed with Kawasaki-like in PIMS-TS with cardiac affliction. Material and method: The study was observational and retrospective, enrolled 14 patients fulfi lling the criteria of PIMS-TS with the median age of 9 (IQR, 1.6-11), 9 male (64.2 %) and 5 female (35.8 %). Results: ECG revealed tachycardia and ST-T changes in 60% of patients. In evolution, ECG modified in 20% and consisted of long QT in 7% of cases, bradycardia in 7%, 3% transitory sick sinus syndrome and 3% grade I/II degree atrio-ventricular block. Cardiac disfunction was evidenced in 4 patients (28%), with reduced ejection fraction under 50%, mitral insuffi ciency in 6 (42.8%), pericardial fl uid in 8 (57.1%) and perivascular brightness in 8 (57.1%). The cardiac biomarkers: NT-proBNP (increased in 9), cTroponin T (incr...
The experience of a favorable outcome of an elective major orthopedic surgical procedure performe... more The experience of a favorable outcome of an elective major orthopedic surgical procedure performed in a severe case of hemophilia A with high titer inhibitors is presented. Specific for the presented case is the minimal dosing schedule of rFVIIa (7 days’ consumption of 68,960 µg vs. standard recommended dosage of 270,000 µg), encouraging for better expectation of success in life-threatening
ABSTRACT Advances in hemophilia care lead to decrease of patients’ mortality and to a better life... more ABSTRACT Advances in hemophilia care lead to decrease of patients’ mortality and to a better life expectancy. These improvements raise another problem: patients have to face with supplementary difficulties related to the disease and its treatment, which can generate emotional reactions.
SummaryA number of 513 consecutive patients (494-haemophilia A and 19-haemophilia B) from eight h... more SummaryA number of 513 consecutive patients (494-haemophilia A and 19-haemophilia B) from eight haemophilia treatment centers have been investigated with Bethesda assay for the presence of factor VIII or IX inhibitors. The overall prevalence of inhibitors was 15.20%, 18.48% in severe, 5.60% in moderate and 12.24% in mild forms. The prevalence was higher than reported in most of the western countries. The age at start of substitution (p = 0.9775), the frequent switching of factor concentrates (p = 0.8931) were not relevant factors for the development of inhibitors. It is worth to be mentioned the unexpectedly occurrence of inhibitors in prior inhibitor negative (6/72) patients (during surgical interventions) probably due to their previous scarce substitution, occurrence which seems not being connected with the continuous infusion modality of factor VIII administration (p = 0.8341). In controversial situations, in the field of low titer (≤1 BU/ml) inhibitors for a reliable interpretat...
SummaryHaemophilia, a lifelong congenital bleeding disease, is a highly demanding disorder, due t... more SummaryHaemophilia, a lifelong congenital bleeding disease, is a highly demanding disorder, due to the costs of its replacement therapy. In the absence of this pivotal treatment, life expectancy and quality of life are deleteriously affected.As illustration, we present a 14 years long follow- up of a patient with severe haemophilia A, treated sporadically with fresh plasma, cryoprecipitate and factor concentrates, who developed a giant iliopsoas pseudotumor. Since he was an infant, under on demand therapy with fresh frozen plasma, cryoprecipitate and low doses of factor concentrates he presented many spontaneous bleedings, developing multiple disabling arthropathies. At the age of 14 years, an iliopsoas hematoma occurred, which relapsed several times, developing an iliopsoas pseudotumour. After 5 years, sepsis with Klebsiella was diagnosed. A CT scan revealed fistula between the pseudotumor and the gut. Under antibiotics, the evolution of sepsis improved, but over a period of 10 mon...
A new born child, age: one month, sex: masculin, with acute respiratory distress syndrome, perior... more A new born child, age: one month, sex: masculin, with acute respiratory distress syndrome, perioronasal cyanosis and hypoxemia has been diagnosed (radiological and imagical-CT) with congenital lobar emphysema (CLE) at the level of the right superior lobe. One month after, the new born child has been diagnosed with the aforesaid syndrome, and, now two months old, he has been subjected to a surgical intervention consisting in lobectomy at the level of the right superior lobe. His postsurgical evolution has proved satisfactory with pulmonary reexpansion.
A new born child, age: one month, sex: masculin, with acute respiratory distress syndrome, perior... more A new born child, age: one month, sex: masculin, with acute respiratory distress syndrome, perioronasal cyanosis and hypoxemia has been diagnosed (radiological and imagical – CT) with congenital lobar emphysema (CLE ) at the level of the right superior lobe. One month after, the new born child has been diagnosed with the aforesaid syndrome, and, now two months old, he has been subjected to a surgical intervention consisting in lobectomy at the level of the right superior lobe. His postsurgical evolution has proved satisfactory with pulmonary reexpansion.
Background: The aim of the study was to document cardiovascular clinical findings, cardiac imagin... more Background: The aim of the study was to document cardiovascular clinical findings, cardiac imaging, and laboratory markers in children presenting with the novel multisystem inflammatory syndrome associated with coronavirus disease 2019 (COVID-19) infection. Methods: This real-time internet-based survey has been endorsed by the Association for European Paediatric and Congenital Cardiologists Working Groups for Cardiac Imaging and Cardiovascular Intensive Care. Children 0 to 18 years of age admitted to a hospital between February 1 and June 6, 2020, with a diagnosis of an inflammatory syndrome and acute cardiovascular complications were included. Results: A total of 286 children from 55 centers in 17 European countries were included. The median age was 8.4 years (interquartile range, 3.8–12.4 years) and 67% were boys. The most common cardiovascular complications were shock, cardiac arrhythmias, pericardial effusion, and coronary artery dilatation. Reduced left ventricular ejection fra...
Summary Background: Haemophilia is a congenital disorder of coagulation with high economic burden... more Summary Background: Haemophilia is a congenital disorder of coagulation with high economic burden due to its requirement for an expensive, lifelong replacement therapy, with additional costs for the frequent complications and for the severe handicapping consequences. The objective of this cross-sectional study aimed at giving an insight into the health condition of young haemophiliacs in the absence of a regular prophylactic therapy. Methods: It was conducted on a heterogeneous group of 37 children and adolescents (4–24 years of age), with similar on demand therapeutic regimen, coming from the whole country, focusing on the joint status by using the Haemophila Joint Health Score (HJHS) system and on quality of life (QoL) by using the EQ-5D-3L-Y questionnaire. Results: The results revealed an impressive situation: 70.3 % with chronic arthropathy, 19 % with target joints, 69 % with multiple joint involvement, mainly elbow (41 %) and knee (34 %), joint damage starting in the age group ...
Objective In countries with low factor concentrate consumption, disabling joint disease remains t... more Objective In countries with low factor concentrate consumption, disabling joint disease remains the major morbidity in patients with haemophilia. The objective of the present analysis is to express the trend and profile of invasive orthopaedic interventions in our country with low usage of factor replacement, lacking the prophylaxis program until recent years. Patients and Methods This retrospective descriptive study was conducted in our university centre in Timisoara with long-lasting experience in haemophilia care, which succeeded in developing an exceptionally valuable genuine comprehensive inter-institutional cooperation. This study refers to 115 invasive interventions performed on 97 patients: 83 with haemophilia A, 10 with haemophilia B and 4 with von Willebrand disease in the period of 2001 to 2017; 17 of them had inhibitors, 5 developing inhibitors after the intervention. Results and Discussion The cohort of patients consisted mostly of young adults, aged between 7 and 49 ye...
The development of FVIII/FIX inhibitor alloantibodies represents a severe complication requiring ... more The development of FVIII/FIX inhibitor alloantibodies represents a severe complication requiring specific laboratory evaluation for establishing a life-saving therapy regimen. Our preliminary study aimed at elaborating a laboratory strategy for monitoring the effectiveness of Activated Prothrombin Complex Concentrate (APCC) and recombinant activated factor VII (rFVIIa) in haemophiliacs with inhibitors, by checking the reliability and clinical value of three complementary assays: clotting-time based coagulometry, thrombelastography (TEG) and thrombin generation assay (TGA). The investigations were performed on 7 patients with severe haemophilia A with high titer inhibitors treated for 12 episodes of severe bleedings, 5 of them for surgical interventions. After the administration of bypassing agents (BPAs) the clotting-time based assay brought changes only on prothrombin (p<0.01), potentially signaling a thrombotic risk, without any impact on the global hemostasis. TEG displayed pr...
Idiopathic thrombocytopenic purpura (ITP) in children is a heterogeneous disease from the point o... more Idiopathic thrombocytopenic purpura (ITP) in children is a heterogeneous disease from the point of view of its natural course and pattern of treatment response. Even in uncomplicated ITP, there is a large variation in disease presentation and prognosis. Although in children it is usually a self limited disorder [1], chronicity (evolution longer than 6 months) and life-threatening bleeds are the main challenges of patients with ITP, justifying preoccupations for their prediction and prevention [2].
... Mean personal income per month in our patients was: preschool children-10.5 € (SD = 0), stude... more ... Mean personal income per month in our patients was: preschool children-10.5 € (SD = 0), students-11.32 € (SD = 6.34), university students-35 ... Haemophilia 1998; 4:491-497 Szucs TD,Offner A, Schramm W. Socioeconomic impact of haemophilia care: results of a pilot study. ...
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