2023-10-12 08:36:13
by Vera Martinella
Before the age of 18 a rare event, but between 2 thousand and 5 thousand new cases occur among children in hospital every year in our country, many of which are undiagnosed. Who risks more and why
Thrombosis is considered a rare event among children, estimated at around one case per 100 thousand children, but in the last two decades in our country the number of thromboses diagnosed in pediatric age has progressively increased to the surprising figure of one case per 200 children hospitalized in intensive care of hospitals. How come? And what can be done?
The numbers
Shedding light on the first Italian Registry of Childhood Thrombosis (RITI) created in 2007 thanks to the collaboration of various experts and in which data are presented on the occasion of World Thrombosis Day, celebrated on 13 October, during a conference in Padua. Let’s start with the numbers: do we know how many cases there are every year in Italy in under 18s? One case in 100 thousand per year in the general population under the age of 14 – replies Paolo Simioni, president of the RITI Registry and director of the Department of Medicine of the University of Padua -. However, if hospitalized pediatric patients are considered, the incidence can be estimated with broad approximation at around 2-5 thousand new cases per year, many of which are not diagnosed. This can correspond to a thrombosis rate equal to 10% of hospitalized pediatric patients, particularly in higher intensity care departments.
Why are cases growing?
The incidence data of thrombotic events secondary to hospitalization has increased more than tenfold in the last 20 years as a consequence of the advancement of resuscitation and surgical techniques and the global increase in life expectancy of children suffering from chronic pathologies – clarifies Simioni, director of the Unit of General Medicine at the Thrombotic-Hemorrhagic Department of the University Hospital of Padua -. Furthermore, the clinical-instrumental diagnosis of thrombosis has become more refined and many more cases are identified today than in the past.
Why do children develop thrombosis?
In the pediatric population, the presence of a central venous catheter represents the greatest risk factor (90% in newborns, over 50% in children) – explains the expert -. Then prematurity, serious infections or some chronic diseases can contribute to the onset of thrombosis. The causes of childhood thrombosis are multiple. Furthermore, compared to adults, the risk factors are less known, difficult to recognize and more complex. Only six cases out of 100 are diagnosed within the three hours needed to establish an effective treatment, in 25-30 cases out of 100 the diagnosis is confirmed within 6 hours, but in over 60 out of 100 the diagnosis occurs late, after 24 hours. The causes of cerebral venous thrombosis in newborns, infants and children are often multifactorial and in 95% of cases predisposition is the main triggering factor. The most common causes are: dehydration, head and neck infections and in particular ear infections, mastoids and sinusitis, meningitis, respiratory tract infections, head trauma and neurosurgical operations, the presence of ventricular shunts -peritoneal, iron deficiency and hematological diseases such as anemia, sickle cell anemia, thalassemia, autoimmune hemolytic anemia and paroxysmal nocturnal hemoglobinuria, autoimmune diseases such as Behet’s disease, SLE and antibody syndrome anti-phospholipids, chronic intestinal diseases, thyrotoxicosis, leukemias and lymphomas, primary brain tumors, heart and kidney diseases (nephrotic syndrome and hemolytic-uremic syndrome), drugs such as corticosteroids, chromosomopathies such as Down syndrome and homocystinuria.
Are there any symptoms to look out for?
Cerebral thromboses in children are divided into arterial and venous – replies Lidia Rota Vender, president of ALT Onlus (Association for the Fight against Thrombosis and cardiovascular diseases) –. Arterial ones, in general, cause cerebral ischemic infarction, also called ischemic stroke, which in pediatric age can manifest itself with acute onset symptoms such as: hemiparesis (difficulty in moving a part of the body) or facial asymmetry, speech difficulties, headache, epileptic seizures, confusion, walking instability, visual disturbances. Venous thrombosis generally causes intracranial hypertension, i.e. increased cerebral pressure, and can be associated with cerebral venous infarction. Cerebral hypertension generally manifests itself with headache, vomiting and visual disturbances.
Are there effective prevention strategies?
Certainly the genetic inheritance of thrombophilia influences the risk of thrombosis in childhood, but it is not possible to exactly establish the percentage of risk nor can it be considered as an isolated risk factor – says Simioni -. In the presence of a suspected diagnosis, it is necessary to confirm with different investigations depending on the area involved: ultrasound Doppler if at the level of a limb or an abdominal organ; magnetic resonance imaging with contrast method or angio-CT if the symptoms are of a cerebral nature or at the level of deep vascular districts. In all cases, these tests will show the presence of the thrombus and absence of venous flow downstream.
How to avoid thrombosis in children?
The risk of the event occurring can be reduced when the favorable conditions are known, for example in the face of hereditary genetic conditions or clinical conditions typically associated with thrombosis such as the presence of a vascular catheter – explains the expert -. Primary or secondary prophylaxis measures for thrombosis can also be implemented with new and modern anticoagulant drugs that can be administered orally today even in children. The prognosis of the thrombotic event depends on the location of the thrombosis (cerebral or systemic), the size of the occluded vessel and the tissue damage at the time of diagnosis.
What causes thrombosis?
Thrombosis is a phenomenon caused by an excess tendency of the blood to clot – explains Lidia Rota Vender, specialist in Hematology and Cardiovascular Diseases due to Thrombosis -. It can be genetically determined, in those who know they have a personal or family thrombophilic condition, or transitory, i.e. linked to climatic situations, to acute or chronic inflammatory diseases (such as pneumonia, cystitis, irritable bowel, kidney or gallbladder stones) or to diseases rheumatic type. Thrombosis can occur in the arteries, causing cerebral stroke, myocardial infarction, limb ischemia which can have devastating consequences such as amputation of the affected limb. Or in superficial or deep veins, causing phlebitis or thrombophlebitis or deep vein thrombosis which unfortunately very often releases emboli that reach the lung causing pulmonary embolism.
A conference and the register
On the occasion of World Thrombosis Day, the first national congress on thrombosis in children will be held in Padua on 12 and 13 October with the presentation of RITI data, a research project supported by ALT (Association for the Fight against Thrombosis and Cardiovascular Diseases ) Onlus. The purpose of the Registry was to collect cases of neonatal and pediatric, cerebral and systemic thrombosis, to take an objective picture of the extent of such events in Italy, to study the epidemiology, the methods of diagnosis, therapy and primary and secondary prevention of such events in Italy. The objectives of the RITI were also to evaluate the clinical relevance of the problem, to promote Italian research in this area to improve care for children affected by thrombosis also through the development of dedicated diagnostic and therapeutic protocols, to finally encourage the creation of a network of doctors expert in assisting these young patients with thrombosis. 15 years after its creation, RITI has become one of the largest Thrombosis Registries in older children in the world, counting over a thousand cases already included with very detailed information on the events that occurred, the risk factors that determined them, the therapies carried out and on the clinical outcome concludes the president of RITI Simioni.
October 12, 2023 (modified October 12, 2023 | 07:41)
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