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Biomedical subjects

Asle Hirth

Publications and source records attributed to Asle Hirth.

10 recordsLinked to original sources
PubMed2006

Recommendations for participation in competitive and leisure sports in patients with congenital heart disease: a consensus document.

BACKGROUND: Physical activity is important for patients with congenital heart disease. The aim of this paper is to provide a consensus document for participation in competitive or leisure sport activity in children and adults with congenital heart disease. METHODS: The recommendations are based on expert consensus meetings, personal experience of the contributing authors and an updated review of the literature regarding exercise performance and risk stratification in patients with congenital heart disease. RESULTS: Physical performance and exercise tolerance is close to normal in patients with simple lesions with successful repair or no need for therapy. Most patients with complex lesions have some degree of residual disease, making them less suitable for participation in competitive sport. CONCLUSION: Regular exercise at recommended levels can be performed and should be encouraged in all patients with congenital heart disease. Many can attend sports with no restrictions. Special concern should be given to those patients with a significant ventricular dysfunction or recent history or risk of arrhythmia.

Adult↗
PubMed2005

[Do patients with ventricular septal defect need endocarditis prophylaxis?].

BACKGROUND: Patients with ventricle septal defect are considered at higher risk of infectious endocarditis than the population in general. According to guidelines, these patients should receive prophylactic antibiotics prior to invasive procedures. Recently, several studies have been published that challenge this view. MATERIAL AND METHODS: Based on searches in Medline, we discuss the risk of endocarditis and indication for antibiotic prophylaxis in patients with this defect. RESULTS: Patients with perimembranous and subvalvular defects have higher risk of endocarditis than the population in general. The proportion of patients developing endocarditis in relation to invasive procedures is low. Prophylactic antibiotics are only partially effective. INTERPRETATION: The proportion of preventable cases of endocarditis is low, and the prophylaxis does not give sufficient protection. Studies suggest that only patients at the highest risk should receive prophylactics.

Antibiotic Prophylaxis↗
PubMed2005

[Fluid management and the risk of cerebral oedema in children with diabetic ketoacidosis].

BACKGROUND: Cerebral oedema is a rare but life-threatening complication of diabetic ketoacidosis in children. MATERIAL AND METHODS: This paper presents a case report on an infant with diabetic ketoacidosis and in an extreme hyperosmolar and hypernatremic state. Considerations regarding appropriate fluid management and the osmotic role of serum sodium are discussed. Recommendations for treatment are given based on available guidelines and published evidence. INTERPRETATION: Intracellular swelling of the brain seems to be caused by water moving along osmotic gradients. In diabetic ketoacidosis, sodium and glucose are the dominant osmotic forces. It is important to understand the relation between serum osmolarity, sodium and glucose, as appropriate fluid treatment might prevent this potentially fatal complication.

Blood Glucose↗
PubMed2005

[Evaluation of cardiac murmurs in children].

BACKGROUND: Many normal children have heart murmurs, but most children do not have heart disease. Differentiation of innocent murmurs from those due to structural heart disease, pathological murmurs, is largely clinical. MATERIAL AND METHODS: This review is based upon the authors' own studies and PubMed searches. RESULTS: An appropriate history and a properly conducted physical examination can identify children at risk of significant heart disease. INTERPRETATION: If a murmur cannot clearly be labelled as innocent based on characteristics like sound quality, intensity, location and response to posture, then referral to a paediatric cardiologist is indicated. Other indications for referral are any sign or symptom of cardiovascular disease such as shortness of breath, cyanosis or decreased exercise tolerance. In addition, children with syndromes should, because of their high risk of congenital heart disease, be referred to a paediatric cardiologist for further evaluation.

Child↗
PubMed2003

[Intravenous enzyme substitution therapy in children with Fabry's disease].

BACKGROUND: Fabry's disease is a X-linked lysosomal storage disorder with accumulation of globotriaosylceramide primarily in vascular endothelial cells, affecting mainly skin, kidney, heart and brain. Symptoms may appear in 7-8-year-old children as burning pain in hands and feet; organ damage usually becomes evident in adult age. Intravenous enzyme replacement therapy has recently become available. PATIENTS: Two brothers, 13.5 and 15 years old, had typical symptoms with acroparesthesia, fever, abdominal pain and diarrhoea. There were high levels of urinary globotriaosylceramide, low levels of alfagalactosidase A in leukocytes, and a single-nucleotide deletion in position 10671 of the alfagalactosidase gene (GLA). Both brothers showed slightly abnormal echocardiography and one had typical changes on cerebral MRI. Enzyme replacement therapy with alfagalactosidase A (Replagal) was started in November 2001. After one year of therapy there was clearly clinical improvement with no fever and diarrhoea and less pain. The urinary excretion of globotriaosylceramide was reduced. No adverse effects were recorded. INTERPRETATION: During 12 months of intravenous enzyme replacement therapy in two adolescent subjects with Fabry's disease we observed clinical improvement and no adverse effects. We recommend greater awareness of Fabry's disease in paediatrics as well as in adult medicine.

Adolescent↗
PubMed2003

Gamma-knife radiosurgery in pediatric cerebral and skull base tumors.

BACKGROUND: This retrospective study of 12 children with cerebral or skull base tumors was undertaken to evaluate morbidity and outcome after gamma-knife surgery. PROCEDURE: Twelve consecutive children treated with stereotactic radiosurgery in a curative intent were reviewed. There were five girls and seven boys. The mean age at diagnosis was 5.8 years and at radiosurgical treatment 8.4 years. There were four pilocytic astrocytomas, two craniopharyngeomas, two pineoblastomas, two ependymomas, and two other tumors of high malignancy. We used a 201-source Co60 Leksell gamma knife and all children were treated in general anesthesia. RESULTS: The mean tumor volume was 3.7 cm(3) and the mean tumor margin dose was 13.8 Gy. Seven patients remained stable after gamma-knife treatment with a mean follow- up of 78.6 months. One patient died during follow-up. The remaining four patients had progressive disease, two within and two outside the irradiated field, and have received further treatment. They are still alive with and without disease with a mean follow-up of 96.8 months. CONCLUSION: Gamma-knife surgery is an effective treatment in some non-resectable cerebral and skull base pediatric tumors. In most cases, it is used in combination with other therapeutic modalities. It is safe and well tolerated.

Adolescent↗
PubMed2003

Cerebral atypical teratoid/rhabdoid tumor of infancy: long-term survival after multimodal treatment, also including triple intrathecal chemotherapy and gamma knife radiosurgery--case report.

Cerebral atypical teratoid/rhabdoid tumors (AT/RT) of infancy are highly malignant and have a poor prognosis. The authors report on one case with long-term survival. The patient was a 1 year-old boy presenting with a large AT/RT in the right temporal lobe. He was treated with complete surgery, followed by multiagent chemotherapy. Later he had a second resection and intrathecal chemotherapy and Gamma knife radiosurgery was added to the treatment. Except for a well-controlled temporal epilepsy, the boy is doing well after 6 years follow-up. AT/RT should be treated in a multimodal way. Intrathecal chemotherapy and Gamma knife radiosurgery of single recurrent or residual tumors might increase survival.

Antineoplastic Agents↗