Helen Savoia

Hemostasis in neonates and children: Pitfalls and dilemmas

Developmental Hemostasis refers to the age-related changes in the coagulation system that are most marked during neonatal life and childhood. An understanding of these changes is crucial to the accurate diagnosis of hemostatic abnormalities in neonates and children. This paper explains the current understanding of developmental hemostasis and describes the common pitfalls observed in clinical practice through failure to implement the principles into routine diagnostic work. Finally, there is a brief discussion as to a potential physiological rationale for developmental hemostasis and the implications of this for hemostatic interventions in neonates and children. There remains a need for further study to improve our understanding of the implications of developmental hemostasis in normal growth and development.

Venous thromboembolic disease: a single-centre case series study.

Aim:  The epidemiology of venous thromboembolism in children has likely changed since first being described a decade ago because of evolving management strategies and a greater awareness of predisposing factors for thrombosis in children. The Royal Childrens Hospital commenced a 4‐year prospective registry of venous thrombosis in 1999 to determine the current Australian epidemiology of venous thrombosis in infants and children.

Helicobacter pylori infection in the Australian community: current prevalence and lack of association with ABO blood groups

Aims: To assess the current prevalence of Helicobacter pylori infection in an Australian urban population sample and to relate this to age, gender and ABO and Rhesus blood groups.

Arterial thromboembolic disease: A single-centre case series study

Aim:  Paediatric venous thromboembolic disease has been reported with increased frequency during the last decade. In contrast, the pathophysiology of arterial thromboembolic disease in infants and children has not been adequately explored. The aim of this study was to determine the prevalence, aetiology, diagnostic criteria, management and outcome of arterial thromboembolism (TE) in a tertiary paediatric centre.

A review of pathophysiology and current treatment for neonatal alloimmune thrombocytopenia (NAIT) and introducing the Australian NAIT registry

Fetomaternal or neonatal alloimmune thrombocytopenia (NAIT) is a rare but serious condition associated with significant fetal and neonatal morbidity and mortality. The most useful predictor of severe disease is a history of a sibling with an antenatal intracranial haemorrhage. However, NAIT can occur during the first pregnancy and may not be diagnosed until the neonatal period. Antenatal treatment options include maternal intravenous immunoglobulin (IVIG) and corticosteroid treatment, fetal blood sampling (FBS) and intrauterine platelet transfusion (IUT) and early delivery. FBS (with or without IUT) can be used to direct and monitor response to therapy, and to inform mode and timing of delivery. However, this procedure is associated with significant risks, including fetal death, and is generally now reserved for high‐risk pregnancies. This review highlights the current understanding of the epidemiology and pathophysiology of NAIT and summarises current approaches to investigation and management. It also introduces the newly established Australian NAIT registry. Owing to the relative rarity of NAIT, accruing sufficient patient numbers for studies and clinical trials at an institutional level is difficult. This national registry will provide an opportunity to collect valuable information and inform future research on this condition.

Assessing the outcome of systemic tissue plasminogen activator for the management of venous and arterial thrombosis in pediatrics.

This study sought to ascertain the outcomes of systemic thrombolytic therapy used in a cohort of infants and children. Complete thrombus resolution was achieved in 81% of patients with arterial thromboses (n=16) compared to 0% of children with venous thromboses (n=10). A major bleeding rate of 11.5% occurred across the entire cohort (n=3, all arterial). In our cohort, no patient with venous thromboembolism achieved complete resolution of their thrombosis after thrombolytic therapy. More cohort studies reporting the outcome of uniform protocols of thrombolytic therapy in children are required.

An audit of transfusion of red blood cell units in pediatric anesthesia

Background:  Patients undergoing surgery are an important user of red blood cells (RBC). Increasingly, medical staff and patients wish to know the likelihood of RBC transfusion for appropriate resource allocation and to inform preoperative discussions regarding risk. Although some adult data are available, little is known about RBC use in children.

Neonatal exchange transfusions in the 21st century: A single hospital study

In the 21st century, neonatal exchange transfusions (ETs) are uncommon procedures usually performed in tertiary neonatal units. As junior clinical staff now lack familiarity with the procedure, it is important to maintain awareness of its complications in order to manage clinical risks and counsel parents appropriately. The study aims to analyse the ET rate, its indications and its associated complications, in a single tertiary centre in the 21st century.

IVIG – Is it the answer? Maternal administration of immunoglobulin for severe fetal red blood cell alloimmunisation during pregnancy: A case series

Intrauterine transfusion (IUT) is the therapy of choice for fetal anaemia secondary to fetal red blood cell alloimmunisation. Although now standard practice for the anaemic fetus, IUT still confers significant fetal risk. Complication rates of up to 9% and fetal mortality rates of up to 5% have been reported. Particularly challenging is early-onset fetal anaemia with fetal morbidity secondary to IUT reportedly highest in those at mid-gestation. This relates to both increased technical challenges of IUT at early gestations as well as reduced capacity of the premature anaemic and ⁄ or hydropic fetus to adapt to acute correction of its anaemia. The tendency for alloimmunisation to occur ten weeks earlier and to worsen with each subsequent incompatible pregnancy, the technical difficulty at early gestations, and the potential morbidity associated with IUT have prompted the search for alternative treatment modalities. One such alternative is intravenous immunoglobulin (IVIG). Despite proven application in haematology and obstetric medicine, including its antenatal and postnatal use in platelet alloimmunisation, the role of IVIG, albeit promising in fetal red blood cell alloimmunisation, is yet to be fully determined. The objective of this case series was to report the maternal and perinatal outcomes in at risk pregnancies from a single tertiary level obstetric hospital during 2004–2007. In this setting, IVIG was used to eliminate or delay the need for IUT and to reduce overall IUT requirements in women who were at high risk for early-onset fetal red blood cell alloimmunisation.

Hospitalisations for sickle-cell disease in an Australian paediatric population.

Sickle‐cell disease (SCD) is more prevalent in Australia due to increased migration; however, the Australian paediatric SCD population has not been previously described. This study aimed to identify the demographic features of and quantify the hospital resource utilisation in the SCD population at The Royal Childrens Hospital in Victoria.