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P Monagle

Publications and source records attributed to P Monagle.

At least 19 recordsLinked to original sources
PubMed2006

Delayed diagnosis of congenital factor IX deficiency (Christmas disease) in a girl with Turner's Syndrome.

Patients with Turner's syndrome are at risk of X-linked recessive disorders. We report a case of a young girl with Turner's syndrome with persistent mildly abnormal coagulation studies associated with a mild to moderate bleeding diathesis. The abnormalities were initially attributed to intrahepatic cholestasis and were partially responsive to vitamin K. After an interval of several years an episode of unexplained iron deficiency anaemia prompted re-investigation of the mild coagulopathy. Disproportionate reduction in the factor IX concentration and restoration of haemostasis with factor IX concentrate lead to a revised provisional diagnosis of mild haemophilia B which was subsequently confirmed by sequencing the factor IX gene.

Anemia↗
PubMed2004

Neonatal thrombosis and its treatment.

Thromboembolic disease (TE) has been described as the new epidemic of tertiary paediatrics, and no where is this more evident than in the neonatal population. As survival of premature and sick newborns has improved, the frequency of complications associated with intensive supportive therapy and monitoring has increased. Clinically significant thrombosis is emerging as one of the more common complications associated with improved neonatal outcome. The long-term implications of neonatal thrombosis are only just being realised. This systematic review will consider the epidemiology, diagnostic strategies, and outcome for both arterial and venous TE in neonates. The role of inherited thrombophilic abnormalities, and the evidence for anticoagulation therapy will also be considered. The lack of high level evidence in determining optimum therapy is obvious. Further research regarding diagnostic strategies, and optimal therapies is urgently needed.

Catheterization, Peripheral↗
PubMed2004

Arterial ischaemic stroke in children.

OBJECTIVE: Arterial ischaemic stroke (AIS) in childhood is a serious disorder about which little is published. The aim of this study is to determine the epidemiology and outcome of AIS in Australian children. METHODS: Cases of childhood AIS occurring at the Royal Children's Hospital, Melbourne 1993-2001, were identified by medical record search using International Classification of Disease Codes. Information was collected on demographics, risk factors, arterial distribution, results of thrombophilic testing, management and outcome. RESULTS: During the 8 years of review 95 patients presented with 98 cases of AIS calculating an incidence of 1.8 per 100000 children per year. Children less than 12 months of age represented greater than one third of all cases. Identifiable risk factors were present in 64% of cases with congenital heart disease the major risk factor. Thrombophilic testing was incomplete with initial abnormalities present in 18% of cases tested. The estimated stroke-related mortality was 8.4%. Of the patients who survived and who had follow-up details available, 78% had a neurological deficit. Twenty-six patients (26%) received anticoagulation. There was no statistically significant association between treatment with anticoagulation and normal neurological outcome. CONCLUSION: AIS is over-represented in children under 12 months of age and results in death or residual neurological impairment in the majority of cases. Further prospective studies are needed to identify risk factors for poor outcome. The recently established Australian and New Zealand Stroke and Thrombophilia Registry should provide important information on clinical and laboratory based risk factors and create a basis for international clinical trials to improve the outcome of childhood AIS.

Adolescent↗
PubMed2003

Velcroholism.

Explore the source record for details and available documents.

Anemia, Iron-Deficiency↗
PubMed2003

Venous thrombosis in children.

Venous thromboembolic (VTE) events are being increasingly diagnosed in systemic and cerebral vessels in children. Systemic VTE are increasing in children as a result of therapeutic advances and improved clinical acumen in primary illnesses that previously caused mortality. The epidemiology of systemic VTE has been studied in international registries. In children older than 3 months, teenagers are the largest group developing VTE. The most common etiologic factor is the presence of central venous lines. Clinical studies have determined the most sensitive diagnostic method for diagnosing upper system VTE are ultrasound for jugular venous thrombosis and venography for intrathoracic vessels. However, the most sensitive diagnostic methods for lower system VTE and pulmonary embolism (PE) have not been established. Treatment studies for VTE consist of inadequately powered randomized controlled trials or prospective cohort studies. The long-term outcome of systemic VTE, post-thrombotic syndrome, has been reported in children. Cerebral sinovenous thrombosis (CSVT) is becoming increasingly diagnosed in children due to the recognition of the associated subtle clinical symptoms and improved cerebrovascular imaging. The etiology of CSVT includes thrombophilia, head and neck infections, and systemic illness. Estimates of the incidence and outcome of childhood CSVT have recently become available through the Canadian Pediatric Ischaemic Stroke Registry. Clinical studies have not yet been carried out in children to determine the best method of diagnosis or treatment. There have only been case-series studies carried out in the treatment of CSVT. Properly designed clinical trials are urgently required in children with systemic VTE/PE and CSVT to define the best methods of diagnosis, treatment and long-term management.

Adolescent↗
PubMed2003

General anaesthesia or conscious sedation for painful procedures in childhood cancer: the family's perspective.

BACKGROUND: Until recently, midazolam sedation was routinely used in our institution for bone marrow aspirates and lumbar punctures in children with cancer. It has been perceived by many doctors and nurses as being well tolerated by children and their families. AIM: To compare the efficacy of inhalational general anaesthesia and midazolam sedation for these procedures. METHODS: A total of 96 children with neoplastic disorders, who received either inhalational general anaesthesia with sevoflurane, nitrous oxide, and oxygen (GA) or sedation with oral or nasal midazolam (SED) as part of their routine preparation for procedures were studied. The experiences of these children were examined during their current procedure and during their first ever procedure. Main outcome measures were the degree of physical restraint used on the child, and the levels of distress and pain experienced by the child during the current procedure and during the first procedure. The family's preference for future procedures was also determined. RESULTS: During 102 procedures under GA, restraint was needed on four occasions (4%) when the anaesthetic mask was first applied, minimal pain was reported, and children were reported as distressed about 25% of the time. During 80 SED procedures, restraint was required in 94%, firm restraint was required in 66%, the child could not be restrained in 14%, median pain score was 6 (scale 0 (no pain) to 6 (maximum pain)), and 90% of the parents reported distress in their child. Ninety per cent of families wanted GA for future procedures. Many families reported dissatisfaction with the sedation regime and raised concerns about the restraint used on their child. CONCLUSIONS: This general anaesthetic regime minimised the need for restraint and was associated with low levels of pain and distress. The sedation regime, by contrast, was much less effective. There was a significant disparity between the perceptions of health professionals and those of families with respect to how children coped with painful procedures.

Adolescent↗
PubMed2002

Sedation practices for Australian and New Zealand paediatric oncology patients.

OBJECTIVES: Paediatric oncology patients often require repeated bone marrow aspirates and lumbar punctures. These procedures commonly require sedation and analgesia. The Australian and New Zealand College of Anaesthetists and the American Academy of Pediatrics have published guidelines that provide recommendations on monitoring and staffing requirements during sedation of paediatric patients. A survey was conducted of the oncology units in Australia and New Zealand in order to compare current practices with published guidelines. METHODS: Telephone interviews were conducted with nursing or medical staff members. RESULTS: Fourteen oncology units collectively perform approximately 130 procedures each week, of which 74% are performed under general anaesthesia. Of the remainder, most are performed using conscious sedation. Most units adhere to published recommendations regarding equipment and staffing during procedures performed under sedation. Only a minority of units follow guidelines regarding documentation; fasting requirements; observation and documentation of vital signs during and after the procedure; and obtaining informed consent for procedures performed using sedation. CONCLUSION: Sedation practices among paediatric oncology units in Australia and New Zealand vary. None of the units fully adhere to published guidelines on childhood sedation. Paediatric oncology units should be familiar with the content of these guidelines and make an informed decision as to their usefulness, both in directing best clinical practice, and in supporting current practice in the event of medico-legal challenge.

Analgesics↗
PubMed2002

Post-thrombotic syndrome.

Post-thrombotic syndrome (PTS) is a potentially disabling complication occurring in up to 67% of adult patients following deep venous thrombosis (DVT). PTS has recently been recognised in children. We present three cases of symptomatic PTS in children, which occurred following the use of central venous lines (CVLs). In two cases, no symptoms of acute thrombosis were noted. The cases highlight the clinical presentation of this syndrome. A review of the literature revealed two reports describing PTS occurring in children following DVT with an estimated incidence of 7-12%. It is concluded that PTS is an important complication of DVT in children. The clinical findings of pain, swelling, and brawny induration are similar to adult patients. The effect on growing limbs is not known. Paediatricians should be aware of the potential of PTS in all children who are at risk of DVT, including patients with malignancy, congenital heart disease, and children who have had previous CVLs, even in the absence of documented acute DVT.

Adolescent↗
PubMed2001

Thromboembolic disease and antithrombotic therapy in newborns.

This update uses an evidence based approach to analyze and present the epidemiology of neonatal thrombosis, etiologies, currently used techniques for diagnosis with their limitations, and current therapeutic approaches. In addition, the approaches to both prevention and optimal therapies are discussed. In Section I Dr. Paul Monagle addresses the epidemiology of neonatal thrombosis outside of the central nervous system in both arterial and venous locations, and those that occur in utero. The specific contributions of catheters and congenital prothrombotic disorders are delineated. Dr. Monagle also describes currently used techniques for the diagnosis of thrombotic events as well as their limitations and the current therapeutic approaches. In Section II, Dr. Gabrielle deVeber reviews the epidemiology of neonatal thrombosis within the central nervous system, in both arterial and venous locations and those that occur in utero. The neurological presentation, risk factors including congenital prothrombotic disorders, anatomical distribution, diagnostic tests, use of antithrombotic therapy and neurologic outcome of neonates with either sinovenous thrombosis or arterial ischemic stroke are discussed. In Section III, Dr. Anthony Chan reviews the current approaches to the prevention and treatment of neonatal thrombosis. Information on the differences in the response of neonates compared to adults to antithrombotic therapy and new approaches to the prevention and treatment of thrombosis in neonates are emphasized.

Female↗
PubMed2000

The use of low molecular weight heparin in pediatric patients: a prospective cohort study.

OBJECTIVE: Low molecular weight heparins (LMWHs) offer several advantages over standard anticoagulant therapy (unfractionated heparin/warfarin) including predictable pharmacokinetics, minimal monitoring, and subcutaneous administration. Our objective was to determine the safety and efficacy of LMWHs in children. METHODS: A prospective cohort of children treated with the LMWH enoxaparin (Rhone Poulenc Rorer) was monitored at the Hospital for Sick Children, Toronto, Canada, from March 1994 until July 1997. RESULTS: There were 146 courses of LMWH administered for treatment and 31 courses for prophylaxis of thromboembolic events (TEs). Clinical resolution of TEs occurred in 94% of children receiving therapeutic doses of LMWH, and 96% of children receiving prophylactic doses of LMWH had no symptoms of recurrent or new TEs. Major bleeding occurred in 5% of children receiving therapeutic doses. Recurrent or new TEs occurred in 1% and 3% of children receiving therapeutic and prophylactic doses of LMWH, respectively. CONCLUSION: LMWH appears to be efficacious and safe for both management and prophylaxis of TEs. The results of this cohort study justify a randomized controlled trial comparing LMWH with standard therapy for the management of TEs in children.

Adolescent↗
PubMed2000

Outcome of pediatric thromboembolic disease: a report from the Canadian Childhood Thrombophilia Registry.

The outcome for children with deep vein thrombosis (DVT) and pulmonary embolism (PE) is unknown. An understanding of morbidity and mortality of DVT/PE is crucial to the development of rational treatment protocols. The Canadian Childhood Thrombophilia Registry has followed 405 children aged 1 mo to 18 y with DVT/PE for a mean of 2.86 y (range, 2 wk to 6 y) to assess outcome. The all-cause mortality was 65 of 405 children (16%). Mortality directly attributable to DVT/PE occurred in nine children (2.2%), all of whom had central venous line-associated thrombosis. Morbidity was substantial, with 33 children (8.1%) having recurrent thrombosis, and 50 children (12.4%) having postphlebitic syndrome. Recurrent thrombosis and postphlebitic syndrome were more common in older children, although deaths occurred equally in all age groups. The incidence of recurrent thrombosis and postphlebitic syndrome are likely underestimated because of difficulties in diagnosis, especially in younger children. The significant mortality and morbidity found in our study supports the need for international multicenter randomized clinical trials to determine optimal prophylactic and therapeutic treatment for children with DVT/PE.

Adolescent↗
PubMed1999

Spontaneous arterial thrombosis in children.

Spontaneous peripheral artery thrombosis in children is rare. We present 2 cases, in both of which the diagnosis was delayed. Acute arterial insufficiency should be considered in children who have clinical symptoms of leg pain, pallor, and reduced pulses. Angiography is the gold standard to confirm or exclude the diagnosis.

Aspirin↗