Diagnosis and Management of Acquired von Willebrand Disease in Heart Disease: A Review of the Literature.

The incidence of acquired von Willebrand syndrome (AvWS) in patients with heart disease is commonly perceived as rare. However, its occurrence is underestimated and underdiagnosed, potentially leading to inadequate treatment resulting in increased morbidity and mortality.In patients with cardiac disease, AvWS frequently occurs in patients with structural heart disease and in those undergoing mechanical circulatory support (MCS).The clinical manifestation of an AvWS is usually characterized by apparent or occult gastrointestinal (GI) or mucocutaneous hemorrhage frequently accompanied by signs of anemia and/or increased bleeding during surgical procedures. The primary change is loss of high-molecular weight von Willebrand factor multimers (HMWM). Whereas the loss of HMWM in patients with structural heart disease is caused by increased HMWM cleavage by von Willebrand factor (vWF)-cleaving protease, ADAMTS13, AvWS in MCS patients is predominantly a result of a high shear stress coupled with mechanical destruction of vWF itself.This manuscript provides a comprehensive review of the evidence regarding both diagnosis and contemporary management of AVWS in patients with heart disease.

Clinical scoring systems in predicting health-related quality of life of children with injuries.

The aim of the study was to explore the association between Glasgow Coma Scale (GCS), Paediatric Index of Mortality (PIM2) and Injury Severity Score (ISS), and the long-term outcome of children with injuries. The health related quality of life (HRQL) was assessed by using the Royal Alexandra Hospital for children Measure of Function (RAHC MOF), 12 months post discharge. Out of 118 children with injuries (9% of all patients), 75 had injury of the head as the leading injury. There were no significant differences at admission in the severity of clinical condition, as expressed by PIM2 and ISS, between patients with head injuries and patients with other injured leading body regions. Children with head injuries had significantly worse HRQOL than children with other leading injured body region (p < 0.045), and children from road traffic accidents had significantly worse HRQL (p = 0.004), compared to other mechanisms of injury. HRQL correlated significantly with GCS (p = 0.027), but not with ISS and PIM2. As the conclusion, among all scoring systems applied, only GCS, which demonstrates severity of head injury, showed significant impact on long-term outcome of injured children.

Long-term quality of life of patients treated in paediatric intensive care unit.

UNLABELLED: The changes in long-term quality of life (QOL) of children treated in paediatric intensive care unit (PICU) were investigated in relation to their QOL before critical illness together with the influence of underlying chronic health condition and severity of illness estimated by Paediatric Index of Mortality 2 on the long-term outcome. This study included 189 children treated in PICU and 179 children from outpatient clinics as controls. QOL was evaluated according to the Royal Alexandra Hospital for Children Measure of Function (RAHC MOF). The long-term QOL in 70 % of children treated in PICU was good, although there was a significant diminution of QOL in children treated in PICU in comparison with their preadmission scores and with the children from outpatient clinics who served as controls (p < 0.001). Severity of illness had a significant impact on children's QOL (p = 0.016) 6 months after treatment in PICU. Twenty-four months after discharge, the RAHC MOF score was still decreased in 19 % of children treated in PICU, and in significantly more patients with a chronic health condition (CHC) treated in PICU, than in their peers from outpatient clinics (p = 0.029). Reduced QOL was significantly more frequent in children with neurodevelopmental disability than in children without CHC 24 months after discharge from PICU (p = 0.013). CONCLUSION: Acute illness has a significant impact both on children with and without CHC after treatment in PICU 6 months after discharge. Twenty-four months after discharge, comorbidity was identified as the decisive factor for diminished QOL in children after PICU treatment.

[Children with injuries treated in hospital emergency departments]. / Djeca s ozljedama lijecena u bolnickim ambulantama hitnog prijma.

The aim of this study was to determine characteristics of injuries of children admitted to the Emergency Department (ED) of University Hospital Split, and also to define the mechanisms of injuries, as well as the type and severity of injuries. We evaluated 3,221 children with injuries treated in the ED of the University Hospital of Split in the period from January to July 2009. The following indicators were analyzed: age, gender, anatomic distribution of injuries, mechanism, Injury Severity Score (ISS) and the need for hospital and intensive care admission. Chi-square and Mann-Whitney tests were used in order to determine statistical relevance of the results. Boys were more often injured than girls (65.6%), and most of the injured children were older than 13 years (41.7%). The majority of patients (96%) had minor injuries (ISS < 10), and only 3.7% of patients were hospitalized. The majority of injuries were caused by falls (71.3%), and limbs were the most frequently injured body region (67.1%). However, road traffic accidents (RTA) required hospitalization more often than any other mechanism (25% of patients), and the leading injury in RTA victims was head injury (38% of patients). Older children were more susceptible to RTAs (64.5%), and the majority of children were injured as passengers in cars (36.4%). Children with head injuries, and those injured in RTAs, were more often hospitalized and more often admitted to intensive care unit than other patients. The most frequently injured body region in children treated in ED are limbs, and the most frequent mechanism of injury is fall. However, the most severe are head injuries, and the majority of severe injuries are caused by RTAs. These data are important for programs of injury prevention.

Functional outcome of children treated in intensive care unit.

OBJECTIVE: Outcome of patients is determined not only by severity of illness index, but also by the impact of patients' preadmission comorbid status. Therefore, we aimed at evaluating the outcome of patients treated in a pediatric intensive care unit, with special focus on the group of children with chronic diseases. METHODS: Data were obtained prospectively and outcome was assessed according to the Pediatric Overall Performance Category scale for 449 patients in a pediatric intensive care unit of the Split University Hospital. Functional performance was assessed as the preadmission score and the discharge score in patients with neurodevelopmental disabilities, patients with other chronic diseases, and those without chronic disease. RESULTS: The discharge functional status was significantly dependent on the preadmission functional status and on predicted mortality. Children with neurodevelopmental disabilities had the significantly worse baseline score and the significantly smaller deterioration of functional morbidity at discharge compared to children with no chronic disease and children with other chronic diseases. CONCLUSIONS: The Pediatric Overall Performance Category scale has proved its applicability in a small intensive care unit, with a heterogeneous population of patients. It should therefore be considered for regular evaluation of health care quality, as a simple and accurate tool. As opposed to other patients, functional status of children with neurodevelopmental disabilities was markedly influenced by their comorbidity. Their preadmission status was worse than the status of other children, and hence could not significantly deteriorate at discharge.

Desfecho funcional de crianças tratadas em unidade de terapia intensiva / Functional outcome of children treated in intensive care unit

OBJETIVO: O desfecho de pacientes não é somente determinado pelo índice de gravidade de doença, mas também pelo impacto do estado pré-admissão de comorbidade dos pacientes. Portanto, este artigo buscou avaliar o desfecho de pacientes tratados em uma unidade de terapia intensiva pediátrica, com foco especial no grupo de crianças com doenças crônicas. MÉTODOS: Os dados foram obtidos prospectivamente, e o desfecho foi avaliado segundo a escala Pediatric Overall Performance Category para 449 pacientes de uma unidade de terapia intensiva pediátrica do Split University Hospital. O desempenho funcional foi avaliado como o escore pré-admissão e o escore na alta hospitalar em pacientes com alterações neurodesenvolvimentais, com outras doenças crônicas e sem doença crônica. RESULTADOS: O estado funcional à alta hospitalar foi significativamente dependente do estado funcional pré-admissão e da mortalidade prevista. Crianças com alterações neurodesenvolvimentais apresentaram escore basal significativamente pior e deterioração de morbidade funcional na alta hospitalar significativamente menor, comparadas com crianças sem doença crônica e com crianças com outras doenças crônicas. CONCLUSÕES: A escala Pediatric Overall Performance Category demonstrou sua aplicabilidade em uma pequena unidade de terapia intensiva com uma população heterogênea de pacientes. Deve, portanto, ser considerada para avaliação regular de qualidade de cuidados à saúde como uma ferramenta simples e precisa. Ao contrário do que acontece com outros pacientes, o estado funcional de crianças com alterações neurodesenvolvimentais foi marcadamente influenciado por sua comorbidade. Seu estado pré-admissão foi pior do que o de outras crianças e, por isso, não poderia estar significativamente deteriorado na alta hospitalar.

OBJECTIVE: Outcome of patients is determined not only by severity of illness index, but also by the impact of patients' preadmission comorbid status. Therefore, we aimed at evaluating the outcome of patients treated in a pediatric intensive care unit, with special focus on the group of children with chronic diseases. METHODS: Data were obtained prospectively and outcome was assessed according to the Pediatric Overall Performance Category scale for 449 patients in a pediatric intensive care unit of the Split University Hospital. Functional performance was assessed as the preadmission score and the discharge score in patients with neurodevelopmental disabilities, patients with other chronic diseases, and those without chronic disease. RESULTS: The discharge functional status was significantly dependent on the preadmission functional status and on predicted mortality. Children with neurodevelopmental disabilities had the significantly worse baseline score and the significantly smaller deterioration of functional morbidity at discharge compared to children with no chronic disease and children with other chronic diseases. CONCLUSIONS: The Pediatric Overall Performance Category scale has proved its applicability in a small intensive care unit, with a heterogeneous population of patients. It should therefore be considered for regular evaluation of health care quality, as a simple and accurate tool. As opposed to other patients, functional status of children with neurodevelopmental disabilities was markedly influenced by their comorbidity. Their preadmission status was worse than the status of other children, and hence could not significantly deteriorate at discharge.

Frequency of anticardiolipin, antinuclear and anti-beta2 glycoprotein I antibodies in children with epilepsy.

A high prevalence of epilepsies in specific immunological diseases suggests that the immune system may play a role in the pathogenesis of epilepsy or might be associated with it. In this study the frequency of anticardiolipin antibodies (aCL), antinuclear antibodies (ANA) and anti-beta2-glycoprotein I antibodies (anti-beta2-GPI) in 40 children with epilepsy and in 38 healthy subjects was determined. Positive aCL was found in 3 patients, and anti-beta2-GPI in 1 patient. In control group they were negative. ANA antibodies were negative in both groups. Duration of epilepsy < 1 year was observed in all three patients with positive aCL. No statistically significant difference was found concerning the presence of these antibodies between patients and controls. There was no statistically significant correlation of age, sex, age at the onset of epilepsy, duration of epilepsy, type of epilepsy, seizure frequency or specific antiepileptic medications with the presence of any measured antibodies.

Neurodevelopmental disabilities and quality of life after intensive care treatment.

AIM: To analyze the quality of life after Pediatric Intensive Care Unit (PICU) treatment and compare the differences between quality of life in children who suffer chronic health conditions, and those who do not, post discharge from PICU. METHOD: Quality of life was assessed using the Royal Alexandra Hospital for Children (RAHC) Measure of Function which incorporates measures of domains that delineate severity or impact of disease symptoms, physical disability, mobility, emotional distress, behavior, deviation from normal growth and development, impairment in social rules, and performance at school and in leisure activities. RESULTS: Families of 371 children were contacted for RAHC Measure of Function scoring. The results showed that the majority of children with no chronic condition (88.8%), as well as those with a chronic condition other than neurodevelopmental disability (81.6%) had minimal ongoing health problems and good quality of life. Only 21.4% of children with neurodevelopmental disabilities fell into this category, 39.3% had poor quality of life and 39.3% had fair quality of life. CONCLUSION: The results suggest that the majority of patients discharged from the PICU go on to have a good quality of life. The quality of life of patients with other chronic condition did not differ significantly from children who did not suffer from a chronic condition, with the majority of both groups having minimal residual health problems. Conversely, children with neurodevelopmental disabilities were found to have a higher level of health problems and only a fair quality of life compared with the other two groups.

The influence of chronic health conditions on susceptibility to severe acute illness of children treated in PICU.

OBJECTIVES: Our study aimed to assess differences in the susceptibility to severe acute illness in children with and without chronic health conditions treated in a pediatric intensive care unit (PICU). PATIENTS AND METHODS: Data on age, gender, need for ventilator support, length of stay, as well as other parameters for the Paediatric Index of Mortality (PIM2) score were collected. Data were analyzed and compared across three patient groups: those with a neurodevelopmental disability, those with a chronic condition other than a neurodevelopmental disability, and those with no chronic condition. Reasons for admission of patients were classified according to the Australia and New Zealand Paediatric Intensive Care Registry (ANZPIC Registry) diagnostic codes. In the multidisciplinary, seven-bed, level I PICU of the Split University Hospital, the admission data were collected prospectively for 591 consecutively admitted patients aged

An epidemiological study of orofacial clefts in Croatia 1988-1998.

INTRODUCTION: The objective of this epidemiological study was to assess the incidence at birth of orofacial clefts in Croatia. AIM: The aim of the present study was to analyse the character and incidence of orofacial clefts in Croatia and to compare the data with reports from other countries. MATERIAL AND METHODS: All the material for the epidemiological study was retrieved from the documented files from all the neonatal units and hospitals in Croatia providing surgical treatment. RESULTS: A total of 525,298 livebirths were documented during 11 years (1988-1998); 903 among them with orofacial clefts, 24 (2.7%) of them twins. Sixty (6.6%) infants died between birth and the age of 6 months. The incidence of orofacial clefts during the study period was 1.71 per thousand. When eliminating syndromic clefts, the incidence of non-syndromic clefts was 1.56 per thousand. Analysis of cleft lip with or without cleft palate (CL+/-P) and isolated cleft palate only (CP) revealed their incidence to be 1.05 and 0.66 per 1000, respectively. Of all types of clefting, CL and CLA was found in 17.2%, CL+/-P in 43.9%, CP in 38.2% and atypical facial clefts (AFC) in 0.8% of children. Left-sided clefts were most common (51%), followed by bilateral (30.5%) and right-sided (18.5%) clefts. The male to female ratio was 1.3. CL+/-P predominated in male and CP in female children. In 220 cases (24.4%) orofacial clefts were either associated with other anomalies or the clefts occurred as one feature of a syndrome. CONCLUSION: Data obtained from different sources yielded a cleft incidence of 1.71 per 1000 in Croatia. There were no differences in the incidences of orofacial clefts in comparison with similar data from other European countries.