Intracranial hemorrhage in newborns
Bleeding primary intracerebeloasa
This type of bleeding occurs in premature newborn with an incidence of 12-15% in those aged below 32 weeks gestation, and / or weighing less than 1500 g.
A lower incidence is reported in the newborn period.
Etilogie:
Pathogens include: tunics integrity of vascular abnormalities of the skull, impaired cerebrovascular autoregulation with hypoxic-ischemic injury and bleeding from the matrix along the external germline cerebellum.
Clinical signs:
Almost invariably there is a history of perinatal asphyxia or respiratory distress syndrome. Most times there is a catastrophic damage, with apnea, bradycardia and decreased hematocrit, signs that appear in the first two days of life, up to three weeks.
In term newborn there is usually a history of difficult breech birth, which later develop neurological signs of brainstem compression.
Treatment of choice is symptomatic.
Wednesday, March 9, 2011
Intracranial hemorrhage in newborns Primary subarachnoid hemorrhage
Intracranial hemorrhage in newborns
Primary subarachnoid hemorrhage
On this type of hemorrhage were defined three major syndromes:
- Grade I, the most common, minor degree of bleeding without clinical signs and is more common in premature;
- Grade II is characterized by seizures in the 2nd day of life, the infant is otherwise good general condition (good kid with seizures), and evolution is normal in 90% of cases;
- Grade II, more rare, massive subarachnoid hemorrhage with rapidly fatal evolution. These infants have often had an injury severe asphyxia, birth trauma sometimes. Few of them have a major vascular lesions such as aneurysms and vascular malformations.
Paraclinical diagnosis help confirm the diagnosis of primary subarachnoid hemorrhage. When performing the lumbar puncture is distinguished cerebrospinal fluid (CSF) bloody. The calls and computed tomography.
Treatment:
Treatment of seizures requires therapy, treatment of hydrocephalus.
Prognosis:
In the absence of association with hypoxic-ischemic injury and trauma, the prognosis is favorable.
Primary subarachnoid hemorrhage
On this type of hemorrhage were defined three major syndromes:
- Grade I, the most common, minor degree of bleeding without clinical signs and is more common in premature;
- Grade II is characterized by seizures in the 2nd day of life, the infant is otherwise good general condition (good kid with seizures), and evolution is normal in 90% of cases;
- Grade II, more rare, massive subarachnoid hemorrhage with rapidly fatal evolution. These infants have often had an injury severe asphyxia, birth trauma sometimes. Few of them have a major vascular lesions such as aneurysms and vascular malformations.
Paraclinical diagnosis help confirm the diagnosis of primary subarachnoid hemorrhage. When performing the lumbar puncture is distinguished cerebrospinal fluid (CSF) bloody. The calls and computed tomography.
Treatment:
Treatment of seizures requires therapy, treatment of hydrocephalus.
Prognosis:
In the absence of association with hypoxic-ischemic injury and trauma, the prognosis is favorable.
Intracranial hemorrhage in newborns Subdural hemorrhage
Intracranial hemorrhage in newborns
Subdural hemorrhage
This type of bleeding is almost exclusively a traumatic injury of the newborn. The incidence is 5-10% of intracranial bleeding. Major bleeding associated with the production factors include: - The relationship between head size and chain diameter genital - Stiff genital canal; - During labor; - Birth handle.
Etiology:
There are three major varieties of subdural hemorrhage: - Dilacerari tentoriale with torn right sinus, vein of Gallen, lateral sinus; - Dilacerari tent of the brain, the inferior sagittal sinus fracture; - Rupture of superficial cerebral veins.
Clinical signs:
Initially, the infant, usually on time, develop a syndrome manifested by Pontin: stupor, coma, ocular deviation, unevenly dilated pupils, pupillary response to light inconstant, impaired breathing, stiffness or opistotonus. Subdural hemorrhage over the cerebral convexity is associated with at least three clinical grades: Grade I-minor degree of bleeding without apparent clinical signs, grade II signs of brain damage can occur, particularly focal seizures that can be and often is associated with hemiparesis, diversion of the eyes or eye hemiparesis, "doll"; Grade III clinical stage could be represented by the appearance of a subdural hemorrhage in the newborn period, with few clinical signs (tachypnea, child suffering), which develops in the next few months chronic subdural effusion
Diagnosis:
In a positive diagnosis of subdural hemorrhage is a very important role paraclinical diagnosis.
Computerized tomography is a non-invasive method of choice. Cranial ultrasound can not detect small hemorrhages located in the posterior fossa cerebral convexity, but it can diagnose a large hemorrhage. Skull X-rays can diagnose and diastase occipital skull fracture
Treatment:
The surgical treatment of this disease is paramount inerventia, in most cases. Convexity cerebral hemorrhage can be decompressed by subdural puncture and by craniotomy and posterior fossa massive hemorrhage may require craniotomy and aspiration of clots.
Complications and prognosis:
The prognosis of infants with major destructions or stick tentoriale brain is stupid. Death occurs in 45% of cases and survivors shows in most of hydrocephalus or other neurologic sequelae. Infants with mild subdural hemorrhage, small, in normal neurological development in 50%. A serious factor is the association with hypoxic ischemic injury.
Subdural hemorrhage
This type of bleeding is almost exclusively a traumatic injury of the newborn. The incidence is 5-10% of intracranial bleeding. Major bleeding associated with the production factors include: - The relationship between head size and chain diameter genital - Stiff genital canal; - During labor; - Birth handle.
Etiology:
There are three major varieties of subdural hemorrhage: - Dilacerari tentoriale with torn right sinus, vein of Gallen, lateral sinus; - Dilacerari tent of the brain, the inferior sagittal sinus fracture; - Rupture of superficial cerebral veins.
Clinical signs:
Initially, the infant, usually on time, develop a syndrome manifested by Pontin: stupor, coma, ocular deviation, unevenly dilated pupils, pupillary response to light inconstant, impaired breathing, stiffness or opistotonus. Subdural hemorrhage over the cerebral convexity is associated with at least three clinical grades: Grade I-minor degree of bleeding without apparent clinical signs, grade II signs of brain damage can occur, particularly focal seizures that can be and often is associated with hemiparesis, diversion of the eyes or eye hemiparesis, "doll"; Grade III clinical stage could be represented by the appearance of a subdural hemorrhage in the newborn period, with few clinical signs (tachypnea, child suffering), which develops in the next few months chronic subdural effusion
Diagnosis:
In a positive diagnosis of subdural hemorrhage is a very important role paraclinical diagnosis.
Computerized tomography is a non-invasive method of choice. Cranial ultrasound can not detect small hemorrhages located in the posterior fossa cerebral convexity, but it can diagnose a large hemorrhage. Skull X-rays can diagnose and diastase occipital skull fracture
Treatment:
The surgical treatment of this disease is paramount inerventia, in most cases. Convexity cerebral hemorrhage can be decompressed by subdural puncture and by craniotomy and posterior fossa massive hemorrhage may require craniotomy and aspiration of clots.
Complications and prognosis:
The prognosis of infants with major destructions or stick tentoriale brain is stupid. Death occurs in 45% of cases and survivors shows in most of hydrocephalus or other neurologic sequelae. Infants with mild subdural hemorrhage, small, in normal neurological development in 50%. A serious factor is the association with hypoxic ischemic injury.
Intracranial hemorrhage in newborns
Intracranial hemorrhage in newborns
* Introduction
* Subdural hemorrhage
* Primary subarachnoid hemorrhage
* Hemorrhage primary intracerebeloasa
Intracranial hemorrhage include hemorrhagic lesions of the nervous system all conditioned by the act of birth (obstetrical trauma), cerebral hypoxia or hemorrhagic syndrome of the newborn. Common cause permanent neurological sequelae.
Intracranial hemorrhage may have different locations and classifying them causing hemorrhage: the germinative matrix and the ventricles, the posterior fossa (subdural hemorrhage), subarachnoid hemorrhage, bleeding in the brain parenchyma. Bleeding in the brain ventricles and germinative matrix is the most common form of intracranial hemorrhage and occurs almost exclusively in premature infant.
Intraventricular hemorrhage
This type of bleeding occurs in approximately 25-30% of children weighing less than 1500 grams and gestational age below 32 weeks. In 50% of cases bleeding occurs in the first days of life and 90% of cases within 4 days postnatally.
Pathophysiology:
The starting point is the matrix of germline subependimara bleeding - place of glial proliferation and neuronal cells in the first two trimesters of pregnancy. At 25 weeks of gestation the majority of neurons in the cortex are formed, axono-dendritic tree is up and begin to form synapses. Germ is rich matrix containing large vessels and blood supply, irregular, with a poor organization of membrane protein or glial support. Bleeding may remain localized in the matrix germination, or it may extend to the lateral ventricles. The size and exact location of bleeding is determined by the age of gestation. In the first trimester of pregnancy bleeding body extends from the head of caudate nucleus, the foramen Monro. With increasing age of gestation bleeding is reabsorbed, that time is completely lost in most cases. In the newborn period may present a residual germ matrix that occasionally can be the origin of ventricular bleeding, among other sources of intraventricular hemorrhage in the newborn period, as the choroid plexus and venous thrombosis. Efficient self-regulation, cerebral blood flow increases with gestation age. Thus, the developing brain is susceptible to ischemia during disturbances both hypotensive and hypertensive bleeding during disturbances. It seems that the choroid plexus hemorrhage is due to the limited capacity of cerebral blood flow autoregulation. Despite matrix primitive germline cells remain active until 32-34 weeks of gestation and is an area with increased vascularization, the risk of haemorrhage is 4-5 days of life.
Etiology:
Intraventricular hemorrhage is caused by a number of factors associated with acute onset of bleeding: too vigorous resuscitation, respiratory distress syndrome, hypoxemia, acidosis, bicarbonate administration, pneumothorax, seizures.
Of intravascular factors include: fluctuations in cerebral blood flow, increased cerebral blood flow, decreased cerebral blood flow, platelet and coagulation disorders, this ductus arteriosus. Other etiologic agents include: apnea, seizures, handling of the newborn, hiperosmotice infusion solutions, and ECMO hypertension, congestive heart failure, pneumothorax, birth.
Vascular factors include: the integrity of the vascular coats, normal involution of germinal matrix vessels, relatively high blood flow to deep brain structures (in the second and third trimester of pregnancy), hypoxic-ischemic insult in germ matrix and vessels.
Extravascular factors include: weak perivascular support vessels germ matrix, this fibrinolytic enzymes, this bleeding diatheses.
Classification: After intraventricular hemorrhage affected area is classified in three grades: Grade I-matrix hemorrhage in germination and / or minimal intraventricular hemorrhage (less than 10% of ventricular area in section parasagitala), grade II intraventricular hemorrhage less than 50% of the surface ventricular, intraventricular hemorrhage grade III more than 50% of the ventricular surface.
Another popular classification: grade I-subependimara hemorrhage, grade II intraventricular haemorrhage without dilatation, grade III intraventricular hemorrhage with dilation, grade IV intraventricular hemorrhage with dilation and parenchyma.
Clinical signs:
Clinical manifestations may occur in the first zilede life from the 4 and even up to the 21th. In 50-75% of cases are silent, although children may be hemorrhage of grade III or IV. Another manifestation is the catrastrofala with impaired general condition, severe respiratory distress, hypotonia, lethargy, shock, convulsions, coma. Usually these children die. Other specific signs of pathology: a bulging fontanelle, hypotonia, excessive sleepiness, thermal instability, apnea, jaundice or excessive pallor.
Diagnosis:
Dagnosticul put on clinical and paraclinical examination. Of Trans ultrasound examinations should be done in the laboratory on the 3rd or 4th life, followed by a 2nd ultrasound to 7 days to establish the extent of bleeding. Moreover, if severe clinical phenomena ultrasound should be performed weekly to monitor ventricular size. Ultrasonography can identify the full spectrum of severity of haemorrhage, major bleeding Destruction isolated parenchymal bleeding lay. Ultrasound can also view the two major complications of intraventricular hemorrhage: periventricular hemorrhagic infarction and posthemoragica ventriculomegalia (periventricular leukomalacia).
Blood picture reveals decreased hematocrit (Ht) and hemoglobin (Hb) to about 75% of children without clinical symptoms, and thrombocytopenia, with prolonged PT and TPT. Acid-base balance is altered in favor of metabolic acidosis. Monitoring of blood gas shows: hypoxemia, and respiratory acidosis hipercarbie. Hyperbilirubinemia is present and the lumbar puncture is distinguished performance liquid proteinorahie bleeding with 1, 5 g% o
Complications May be complicated by intraventricular hemorrhage: germinal matrix destruction with secondary cystic organization, periventricular leukomalacia, hydrocephalus posthemoragica, hemorrhage and periventricular infarction.
Treatment:
Prophylactic treatment is ideal. Primordial is to avoid premature labor and birth, if it can not be avoided is preferred infant in utero transport to a specialized center of Perinatology, because transport of newborn after birth can influence later neuologic status. Administration of betamethasone 48 hours before birth is thought to be direct consequences to decrease the incidence of intraventricular hemorrhage. The resort and magnesium sulfate tocolysis.
Depending on the aggressiveness of therapy also provides intravascular factors: the prevention or correction of major hemodynamic disorders including cerebral flow fluctuations and increased cerebral venous pressure with a special value in reducing the incidence of bleeding. Pharmacological agents used are phenobarbital, indomethacin, vitamin K, fresh frozen plasma Depending on use etamsilatul vascular factors and vitamin E. Depending on the factors utilized extravascular prolactin.
* Introduction
* Subdural hemorrhage
* Primary subarachnoid hemorrhage
* Hemorrhage primary intracerebeloasa
Intracranial hemorrhage include hemorrhagic lesions of the nervous system all conditioned by the act of birth (obstetrical trauma), cerebral hypoxia or hemorrhagic syndrome of the newborn. Common cause permanent neurological sequelae.
Intracranial hemorrhage may have different locations and classifying them causing hemorrhage: the germinative matrix and the ventricles, the posterior fossa (subdural hemorrhage), subarachnoid hemorrhage, bleeding in the brain parenchyma. Bleeding in the brain ventricles and germinative matrix is the most common form of intracranial hemorrhage and occurs almost exclusively in premature infant.
Intraventricular hemorrhage
This type of bleeding occurs in approximately 25-30% of children weighing less than 1500 grams and gestational age below 32 weeks. In 50% of cases bleeding occurs in the first days of life and 90% of cases within 4 days postnatally.
Pathophysiology:
The starting point is the matrix of germline subependimara bleeding - place of glial proliferation and neuronal cells in the first two trimesters of pregnancy. At 25 weeks of gestation the majority of neurons in the cortex are formed, axono-dendritic tree is up and begin to form synapses. Germ is rich matrix containing large vessels and blood supply, irregular, with a poor organization of membrane protein or glial support. Bleeding may remain localized in the matrix germination, or it may extend to the lateral ventricles. The size and exact location of bleeding is determined by the age of gestation. In the first trimester of pregnancy bleeding body extends from the head of caudate nucleus, the foramen Monro. With increasing age of gestation bleeding is reabsorbed, that time is completely lost in most cases. In the newborn period may present a residual germ matrix that occasionally can be the origin of ventricular bleeding, among other sources of intraventricular hemorrhage in the newborn period, as the choroid plexus and venous thrombosis. Efficient self-regulation, cerebral blood flow increases with gestation age. Thus, the developing brain is susceptible to ischemia during disturbances both hypotensive and hypertensive bleeding during disturbances. It seems that the choroid plexus hemorrhage is due to the limited capacity of cerebral blood flow autoregulation. Despite matrix primitive germline cells remain active until 32-34 weeks of gestation and is an area with increased vascularization, the risk of haemorrhage is 4-5 days of life.
Etiology:
Intraventricular hemorrhage is caused by a number of factors associated with acute onset of bleeding: too vigorous resuscitation, respiratory distress syndrome, hypoxemia, acidosis, bicarbonate administration, pneumothorax, seizures.
Of intravascular factors include: fluctuations in cerebral blood flow, increased cerebral blood flow, decreased cerebral blood flow, platelet and coagulation disorders, this ductus arteriosus. Other etiologic agents include: apnea, seizures, handling of the newborn, hiperosmotice infusion solutions, and ECMO hypertension, congestive heart failure, pneumothorax, birth.
Vascular factors include: the integrity of the vascular coats, normal involution of germinal matrix vessels, relatively high blood flow to deep brain structures (in the second and third trimester of pregnancy), hypoxic-ischemic insult in germ matrix and vessels.
Extravascular factors include: weak perivascular support vessels germ matrix, this fibrinolytic enzymes, this bleeding diatheses.
Classification: After intraventricular hemorrhage affected area is classified in three grades: Grade I-matrix hemorrhage in germination and / or minimal intraventricular hemorrhage (less than 10% of ventricular area in section parasagitala), grade II intraventricular hemorrhage less than 50% of the surface ventricular, intraventricular hemorrhage grade III more than 50% of the ventricular surface.
Another popular classification: grade I-subependimara hemorrhage, grade II intraventricular haemorrhage without dilatation, grade III intraventricular hemorrhage with dilation, grade IV intraventricular hemorrhage with dilation and parenchyma.
Clinical signs:
Clinical manifestations may occur in the first zilede life from the 4 and even up to the 21th. In 50-75% of cases are silent, although children may be hemorrhage of grade III or IV. Another manifestation is the catrastrofala with impaired general condition, severe respiratory distress, hypotonia, lethargy, shock, convulsions, coma. Usually these children die. Other specific signs of pathology: a bulging fontanelle, hypotonia, excessive sleepiness, thermal instability, apnea, jaundice or excessive pallor.
Diagnosis:
Dagnosticul put on clinical and paraclinical examination. Of Trans ultrasound examinations should be done in the laboratory on the 3rd or 4th life, followed by a 2nd ultrasound to 7 days to establish the extent of bleeding. Moreover, if severe clinical phenomena ultrasound should be performed weekly to monitor ventricular size. Ultrasonography can identify the full spectrum of severity of haemorrhage, major bleeding Destruction isolated parenchymal bleeding lay. Ultrasound can also view the two major complications of intraventricular hemorrhage: periventricular hemorrhagic infarction and posthemoragica ventriculomegalia (periventricular leukomalacia).
Blood picture reveals decreased hematocrit (Ht) and hemoglobin (Hb) to about 75% of children without clinical symptoms, and thrombocytopenia, with prolonged PT and TPT. Acid-base balance is altered in favor of metabolic acidosis. Monitoring of blood gas shows: hypoxemia, and respiratory acidosis hipercarbie. Hyperbilirubinemia is present and the lumbar puncture is distinguished performance liquid proteinorahie bleeding with 1, 5 g% o
Complications May be complicated by intraventricular hemorrhage: germinal matrix destruction with secondary cystic organization, periventricular leukomalacia, hydrocephalus posthemoragica, hemorrhage and periventricular infarction.
Treatment:
Prophylactic treatment is ideal. Primordial is to avoid premature labor and birth, if it can not be avoided is preferred infant in utero transport to a specialized center of Perinatology, because transport of newborn after birth can influence later neuologic status. Administration of betamethasone 48 hours before birth is thought to be direct consequences to decrease the incidence of intraventricular hemorrhage. The resort and magnesium sulfate tocolysis.
Depending on the aggressiveness of therapy also provides intravascular factors: the prevention or correction of major hemodynamic disorders including cerebral flow fluctuations and increased cerebral venous pressure with a special value in reducing the incidence of bleeding. Pharmacological agents used are phenobarbital, indomethacin, vitamin K, fresh frozen plasma Depending on use etamsilatul vascular factors and vitamin E. Depending on the factors utilized extravascular prolactin.
Haemophilia type A Treatment
Haemophilia type A
Treatment
Treatment consists of preventing and combating hemorrhagic accidents. For this purpose specific substitution factor is deficient according to the duration of its life, all life possible.
Curative treatment of bleeding provides combat casualties reported. Prophylactic therapy includes avoiding high physical exertion. Prophylaxis are provided in compliance with the following contraindications: - Medical maneuvers bleeding: intraarterial, intramuscular hematomas or haemarthroses puncture, cauterization; - Restriction of ENT surgery, dental care (only with special training); - Eviatarea administration of aspirin or nonsteroidal anti-inflammatory (NSAID) that causes bleeding from the gastrointestinal mucosa, in particular; - Tetanus vaccination, BCG, DPT, polio are normal; - Avoid prolonged immobilization as leading to ankylosis; - Avoid circular plaster device; - Limiting hospitalization. Substitution therapy is essential to assessing the haemorrhagic accident: - Light bleeding when factor VIII has a value of 20%; - Average bleeding when factor VIII has a value of 30%; - Severe bleeding when the factor VIII has a value of 40-50%. - Severe bleeding when the factor VIII has a value of 100% and over.
If the amount of clotting time (CT) exceeds 40 minutes, when factor VIII is less than 0. 1%. When the amount of clotting time is in the range 18-40 minutes, factor VIII is the percentage of 1%.
A special importance is given knowledge of factor VIII concentration of the preparation used. Thus, whole blood has a concentration of factor VIII 5. 8UI/ml, is a lyophilized plasma concentration 1UI/ml and cee antihemofilica shows a 2UI/ml concentration of factor VIII, cryoprecipitate has 4UI/ml, vials containing human antihemofilica 25-50UI/ml with globulin, and the animal - 200UI/ml.
Half-life knowledge is required to treatment, whose value is 10-16 hours: 3-4 doses / day and 2 severe informele doze/12 hours later. Fever is known to increase the need for vitamin D. Prevention of isoimmunization is the intra-venous infusion 15-30 minutes fast. Factor VIII transfusion requirements 1UI / kg increased by 2% concentration of factor VIII in medium forms - the factor VIII dose is administered 20UI/kgc necessary.
Control bleeding if haemarthroses 1-3 provides for the transfusion factor VIII if the existence of muscle hematomas are suficienete 1-2 transfusions, fecal nerve compression transfusions are 3-6, 3-6 retroperitoneal-transfusion-retropharyngeal 3-6 transfusions, intracranial, 7-14 transfusions. Adjuvant corticosteroid treatment provides for the administration of prednisone at a dose of 0. 5-1mg/kgc/zi per-orally for 3-4 days, when there haemarthrosis, haematuria, bleeding post-dental extractions. Epsilon aminocaproic ACID is administered at a dose of 100-400mg/kgc/zi, if per-oral gingival bleeding, epistaxis, bleeding alveolo-dental (contraindicated in haematuria). It aims to avoid aspirin, indomethacin has, of novocaine, the morphine, papaverine.
Local hemostasis is made by: compression, administration of thrombin and GELASPON ice pack.
It is recommended inteventia surgery, orthopedic surgery, functional rehabilitation and gym. Nosebleeds can be treated locally by performing compression lavage with saline and ten minutes, mesa with thrombin. Substitution therapy is contraindicated cauterization, is used only in extreme cases. Post-extraction bleeding stops by: cleaning the socket, the administration of thrombin GELASPON local, 1-2 make Factor VIII transfusion, Epsilon aminocaproic ACID administration for 10-15 days (protects the clot) and prednisone for a period corticoterpie 3-4 days. Tracking is indicated for 5-9 days (daily) because there is risk of delayed bleeding.
Treatment for hematuria consists of: rest, diuresis cure, substitution treatment.
If haemarthrosis patient is immobilized in physiological position, place ice on the affected joint and is substitution treatment.
Take a series of physical and psychosocial measures: homes 5-10 years, educational and vocational training for professions without exercise, mental health: restrictions limiting school activities (tailored to their abilities) and expansion of home care.
Prophylactic treatment consists 16-32U/kgc/saptamana substitute in 1-3 home doses per week to make a better quality of life, accident avoidance repetitive bleeding, functional sequelae. This treatment provides a number of risks: isoimmunization, in severe cases increased cost factor VIII less than 1% bleeding frequency of 3-4 accidents per month. Bear in mind that genetic counseling is as radical.
Mortality is present in a rate of 3%. Functionality influence prognosis: - Disabling in haemophilia arthropathy leading to chronic stiffness; - Muscle: motor defeat; - Sensing: deafness, blindness; - Hemiplegia, epilepsy.
Disease complications are: - Mechanical compression that causes paralysis, pathological bone fractures; - Iatrogenic - HIV, HBV, HCV circulating antibodies against factor VIII (10-20%), posttransfuzionale.
Treatment
Treatment consists of preventing and combating hemorrhagic accidents. For this purpose specific substitution factor is deficient according to the duration of its life, all life possible.
Curative treatment of bleeding provides combat casualties reported. Prophylactic therapy includes avoiding high physical exertion. Prophylaxis are provided in compliance with the following contraindications: - Medical maneuvers bleeding: intraarterial, intramuscular hematomas or haemarthroses puncture, cauterization; - Restriction of ENT surgery, dental care (only with special training); - Eviatarea administration of aspirin or nonsteroidal anti-inflammatory (NSAID) that causes bleeding from the gastrointestinal mucosa, in particular; - Tetanus vaccination, BCG, DPT, polio are normal; - Avoid prolonged immobilization as leading to ankylosis; - Avoid circular plaster device; - Limiting hospitalization. Substitution therapy is essential to assessing the haemorrhagic accident: - Light bleeding when factor VIII has a value of 20%; - Average bleeding when factor VIII has a value of 30%; - Severe bleeding when the factor VIII has a value of 40-50%. - Severe bleeding when the factor VIII has a value of 100% and over.
If the amount of clotting time (CT) exceeds 40 minutes, when factor VIII is less than 0. 1%. When the amount of clotting time is in the range 18-40 minutes, factor VIII is the percentage of 1%.
A special importance is given knowledge of factor VIII concentration of the preparation used. Thus, whole blood has a concentration of factor VIII 5. 8UI/ml, is a lyophilized plasma concentration 1UI/ml and cee antihemofilica shows a 2UI/ml concentration of factor VIII, cryoprecipitate has 4UI/ml, vials containing human antihemofilica 25-50UI/ml with globulin, and the animal - 200UI/ml.
Half-life knowledge is required to treatment, whose value is 10-16 hours: 3-4 doses / day and 2 severe informele doze/12 hours later. Fever is known to increase the need for vitamin D. Prevention of isoimmunization is the intra-venous infusion 15-30 minutes fast. Factor VIII transfusion requirements 1UI / kg increased by 2% concentration of factor VIII in medium forms - the factor VIII dose is administered 20UI/kgc necessary.
Control bleeding if haemarthroses 1-3 provides for the transfusion factor VIII if the existence of muscle hematomas are suficienete 1-2 transfusions, fecal nerve compression transfusions are 3-6, 3-6 retroperitoneal-transfusion-retropharyngeal 3-6 transfusions, intracranial, 7-14 transfusions. Adjuvant corticosteroid treatment provides for the administration of prednisone at a dose of 0. 5-1mg/kgc/zi per-orally for 3-4 days, when there haemarthrosis, haematuria, bleeding post-dental extractions. Epsilon aminocaproic ACID is administered at a dose of 100-400mg/kgc/zi, if per-oral gingival bleeding, epistaxis, bleeding alveolo-dental (contraindicated in haematuria). It aims to avoid aspirin, indomethacin has, of novocaine, the morphine, papaverine.
Local hemostasis is made by: compression, administration of thrombin and GELASPON ice pack.
It is recommended inteventia surgery, orthopedic surgery, functional rehabilitation and gym. Nosebleeds can be treated locally by performing compression lavage with saline and ten minutes, mesa with thrombin. Substitution therapy is contraindicated cauterization, is used only in extreme cases. Post-extraction bleeding stops by: cleaning the socket, the administration of thrombin GELASPON local, 1-2 make Factor VIII transfusion, Epsilon aminocaproic ACID administration for 10-15 days (protects the clot) and prednisone for a period corticoterpie 3-4 days. Tracking is indicated for 5-9 days (daily) because there is risk of delayed bleeding.
Treatment for hematuria consists of: rest, diuresis cure, substitution treatment.
If haemarthrosis patient is immobilized in physiological position, place ice on the affected joint and is substitution treatment.
Take a series of physical and psychosocial measures: homes 5-10 years, educational and vocational training for professions without exercise, mental health: restrictions limiting school activities (tailored to their abilities) and expansion of home care.
Prophylactic treatment consists 16-32U/kgc/saptamana substitute in 1-3 home doses per week to make a better quality of life, accident avoidance repetitive bleeding, functional sequelae. This treatment provides a number of risks: isoimmunization, in severe cases increased cost factor VIII less than 1% bleeding frequency of 3-4 accidents per month. Bear in mind that genetic counseling is as radical.
Mortality is present in a rate of 3%. Functionality influence prognosis: - Disabling in haemophilia arthropathy leading to chronic stiffness; - Muscle: motor defeat; - Sensing: deafness, blindness; - Hemiplegia, epilepsy.
Disease complications are: - Mechanical compression that causes paralysis, pathological bone fractures; - Iatrogenic - HIV, HBV, HCV circulating antibodies against factor VIII (10-20%), posttransfuzionale.
Haemophilia type A Diagnosis
Haemophilia type A
Diagnosis
Positive diagnosis:
To issue a diagnosis of hemophilia correlates the clinical and laboratory data. Clinical symptoms include: bleeding externalized, incoercible, deep hemorrhage (tissue, cavitary, visceral) sometimes extensive.
Laboratory testing reveals efctuate: bleeding time (TS) normal clotting time (CT) increased by performing the test tube, Howell, they are screening tests. It also highlights the prothrombin consumption low, normal is between 40-50 seconds. PTTK PTT and factor VIII increase when less than 20%. Thromboplastin generation test is altered during Quick (TQ), TS, thromboplastin, fibrinolytic activation is normal. During two of the clotting cascade, the conversion of prothrombin into thrombin under the action of thromboplastin time is explored Quick (TQ), it is normal for the thromboplastin is added from outside. The first stage of coagulation, intrinsic and extrinsic tromboplastinoformarea by the PTT and PTTK is explored. Tests of clotting time correction (TC) when fresh plasma is suggestive highlights hemophilia A and plasma factor adsorbed, suggestive of hemophilia B. Factor
Differential Diagnosis
The differential diagnosis is made with: deficiency of factors IX, XII; syndromes parahemofilice deficient in factors V and VII, immune thrombocytopenic purpura (ITP).
Diagnosis
Positive diagnosis:
To issue a diagnosis of hemophilia correlates the clinical and laboratory data. Clinical symptoms include: bleeding externalized, incoercible, deep hemorrhage (tissue, cavitary, visceral) sometimes extensive.
Laboratory testing reveals efctuate: bleeding time (TS) normal clotting time (CT) increased by performing the test tube, Howell, they are screening tests. It also highlights the prothrombin consumption low, normal is between 40-50 seconds. PTTK PTT and factor VIII increase when less than 20%. Thromboplastin generation test is altered during Quick (TQ), TS, thromboplastin, fibrinolytic activation is normal. During two of the clotting cascade, the conversion of prothrombin into thrombin under the action of thromboplastin time is explored Quick (TQ), it is normal for the thromboplastin is added from outside. The first stage of coagulation, intrinsic and extrinsic tromboplastinoformarea by the PTT and PTTK is explored. Tests of clotting time correction (TC) when fresh plasma is suggestive highlights hemophilia A and plasma factor adsorbed, suggestive of hemophilia B. Factor
Differential Diagnosis
The differential diagnosis is made with: deficiency of factors IX, XII; syndromes parahemofilice deficient in factors V and VII, immune thrombocytopenic purpura (ITP).
Haemophilia type A Clinical signs
Haemophilia type A
Clinical signs
In terms of clinical diagnosis is rare in newborn. The increased incidence of disease is between 1-5 years of age. A percentage between 30-40% of cases are diagnosed in adults. Diagnosis may be issued and fortuitously, as a result of bleeding injuries. Thus, 6% of cases are diagnosed in the newborn, infant 8%, 32% in a range of life-2 years and 40% over 2 years old. They do not appear to cut the umbilical cord tissue is increased because trombelastina, occurring in circumcision. The newborn is diagnosed more easily when there is a family history of haemophilia. Features installed hemorrhage: a hemorrhage caused, occurring after minor trauma, shows an increased duration of bleeding externalized; have registered deeply in the cavity of capsules.
The disease has a chronic evolution, with periods of calm. The absence of petechial lesions differential diagnosis helps immune thrombocytopenic purpura, because it affected tissue hemostasis. Intramuscular hematoma show similar clinical Tabolul superficial level buttocks, deltoid, pectoralis, deep in the psoas muscle, retroperitoneal a rate of 10-15%.
Functional prognosis is negative when there is compression of the radial nerve, the sciatic vascular package, sense organs: eye, ear internal functional rebound.
This event in haemophilia haemarthroses that determine the occurrence of ankylosis in chronic evolution, especially in May vicious position. A percentage of 89% of the cases shows haemarthrosis were affected: knee, malleolus, elbows, punches, hip. Of these 50% shows a low severity, 30% are 20% of medium gravity and high gravity.
After the place of occurrence, the bleeding is calsifica: visceral bleeding, bleeding in open-wounds 50%, mouth bleeding, bruises, cut, epistaxis (mucosal), the latter are abundant and have a prolonged time can lead to collapse and anemia. Visceral Bleeding hematuria include severe repetitive, at a rate of 20%, gastrointestinal bleeding, bleeding of the sense organs: eye, inner ear, brain hemorrhage, bleeding after surgery and medical interventions.
Clinical forms of hemophilia are: family form that is found in 60% and sporadic form in 40%.
Depending on the severity of factor VIII, there are several forms of disease: - Severe forms in less than 1% of cases; - Forms a percentage average of 1-4%; - Mild in the rate of 4-25%; - Latent form in a higher percentage of 25%.
Clinical signs
In terms of clinical diagnosis is rare in newborn. The increased incidence of disease is between 1-5 years of age. A percentage between 30-40% of cases are diagnosed in adults. Diagnosis may be issued and fortuitously, as a result of bleeding injuries. Thus, 6% of cases are diagnosed in the newborn, infant 8%, 32% in a range of life-2 years and 40% over 2 years old. They do not appear to cut the umbilical cord tissue is increased because trombelastina, occurring in circumcision. The newborn is diagnosed more easily when there is a family history of haemophilia. Features installed hemorrhage: a hemorrhage caused, occurring after minor trauma, shows an increased duration of bleeding externalized; have registered deeply in the cavity of capsules.
The disease has a chronic evolution, with periods of calm. The absence of petechial lesions differential diagnosis helps immune thrombocytopenic purpura, because it affected tissue hemostasis. Intramuscular hematoma show similar clinical Tabolul superficial level buttocks, deltoid, pectoralis, deep in the psoas muscle, retroperitoneal a rate of 10-15%.
Functional prognosis is negative when there is compression of the radial nerve, the sciatic vascular package, sense organs: eye, ear internal functional rebound.
This event in haemophilia haemarthroses that determine the occurrence of ankylosis in chronic evolution, especially in May vicious position. A percentage of 89% of the cases shows haemarthrosis were affected: knee, malleolus, elbows, punches, hip. Of these 50% shows a low severity, 30% are 20% of medium gravity and high gravity.
After the place of occurrence, the bleeding is calsifica: visceral bleeding, bleeding in open-wounds 50%, mouth bleeding, bruises, cut, epistaxis (mucosal), the latter are abundant and have a prolonged time can lead to collapse and anemia. Visceral Bleeding hematuria include severe repetitive, at a rate of 20%, gastrointestinal bleeding, bleeding of the sense organs: eye, inner ear, brain hemorrhage, bleeding after surgery and medical interventions.
Clinical forms of hemophilia are: family form that is found in 60% and sporadic form in 40%.
Depending on the severity of factor VIII, there are several forms of disease: - Severe forms in less than 1% of cases; - Forms a percentage average of 1-4%; - Mild in the rate of 4-25%; - Latent form in a higher percentage of 25%.
Haemophilia type A
Haemophilia type A
* Introduction
* Clinical signs
* Diagnosis
* Treatment
Haemophilia is transmitted X-linked congenital, hereditary nature. The cause of the disease is represented by synthesis or alteration in quality small amount of globulin antihemofilice A or B. The epidemiological 120-130 are found in 100 cases. 000 births.
Haemophilia type A meets a percentage of 80% of Haemophilia syndromes. This type of disease is 4-5 times more common than type B. Haemophilia Pathogenesis of disease is criminalized in a deficit of factor VIII: C VIII clotting activates itself, VIII R - WF (Willebrand), and VIII RA (antigen). These are two essential fractions: high molecular weight and high molecular weight (factor VIII C).
A percentage of 60% of cases familial disease, inherited by heterozygous mothers (XnormalXpatologic) XpatologicY boys expressing homozygous or, in exceptional cases. A percentage of 40% of cases occur after a mutation or illegitimate children with a history or falsified. The value of the complex physiological FVIII-FVW is to increase the synthesis of FVIII, protecting it from proteolysis and it focuses on places of active hemostasis, specifically in the vascular lesions. This occurs through binding factor subendoteliale FVW vascular matrix proteins and platelets. The main role of FVIII in the coagulation process is to accelerate the rate and rhythm splitting FX, so the latter factor is activated by activation of F IX. In this recatii involved in several components, FVIII enzyme with substrate are focused on a phospholipid surface to form a complex with calcium dependent. Separation of FVW FVIII to bind to the phospholipid surface is by enzymatic cleavage of FVIII by thrombin molecule or F Xa.
In terms of pathogenic, FVIII deficiency tromboplastinogeneza endogenous change, disrupts the conversion of prothrombin into thrombin leading to fibrin formation in insufficient quantities. In patients with haemophilia, with the introduction of polymerase chain reaction (PCR) and DNA study of restriction enzymes have made progress in the studies of mutations in genes affecting the F VIII synthesis. It has been shown as a percentage of 50% of the mutations that cause severe hemophilia type A were created by reversing the DNA sequence in intron 22 FVIII gene that causes disjunction. Actualmenete, specific mutations are used to identify the genes direcete antenatal diagnosis and analysis of carriers of haemophilia. Analysis of these mutations may be useful in determining the prediction of the likely development of antibodies inactivated FVIII or IX. There is a heterogeneity of mutations in hemophilia A, in explaining the diversity in clinical severity of the disease and how manifestrae present in patients with hemophilia A. Genetic defects in hemophilia A consist in joint inversions with the chromosomal material pierderae interseaza intron 22. Major defects are found, such as deletions, duplications, translocations found in 3-5% of cases or small defects, such as different mutations responsible for 75% of cases of hemophilia A.
* Introduction
* Clinical signs
* Diagnosis
* Treatment
Haemophilia is transmitted X-linked congenital, hereditary nature. The cause of the disease is represented by synthesis or alteration in quality small amount of globulin antihemofilice A or B. The epidemiological 120-130 are found in 100 cases. 000 births.
Haemophilia type A meets a percentage of 80% of Haemophilia syndromes. This type of disease is 4-5 times more common than type B. Haemophilia Pathogenesis of disease is criminalized in a deficit of factor VIII: C VIII clotting activates itself, VIII R - WF (Willebrand), and VIII RA (antigen). These are two essential fractions: high molecular weight and high molecular weight (factor VIII C).
A percentage of 60% of cases familial disease, inherited by heterozygous mothers (XnormalXpatologic) XpatologicY boys expressing homozygous or, in exceptional cases. A percentage of 40% of cases occur after a mutation or illegitimate children with a history or falsified. The value of the complex physiological FVIII-FVW is to increase the synthesis of FVIII, protecting it from proteolysis and it focuses on places of active hemostasis, specifically in the vascular lesions. This occurs through binding factor subendoteliale FVW vascular matrix proteins and platelets. The main role of FVIII in the coagulation process is to accelerate the rate and rhythm splitting FX, so the latter factor is activated by activation of F IX. In this recatii involved in several components, FVIII enzyme with substrate are focused on a phospholipid surface to form a complex with calcium dependent. Separation of FVW FVIII to bind to the phospholipid surface is by enzymatic cleavage of FVIII by thrombin molecule or F Xa.
In terms of pathogenic, FVIII deficiency tromboplastinogeneza endogenous change, disrupts the conversion of prothrombin into thrombin leading to fibrin formation in insufficient quantities. In patients with haemophilia, with the introduction of polymerase chain reaction (PCR) and DNA study of restriction enzymes have made progress in the studies of mutations in genes affecting the F VIII synthesis. It has been shown as a percentage of 50% of the mutations that cause severe hemophilia type A were created by reversing the DNA sequence in intron 22 FVIII gene that causes disjunction. Actualmenete, specific mutations are used to identify the genes direcete antenatal diagnosis and analysis of carriers of haemophilia. Analysis of these mutations may be useful in determining the prediction of the likely development of antibodies inactivated FVIII or IX. There is a heterogeneity of mutations in hemophilia A, in explaining the diversity in clinical severity of the disease and how manifestrae present in patients with hemophilia A. Genetic defects in hemophilia A consist in joint inversions with the chromosomal material pierderae interseaza intron 22. Major defects are found, such as deletions, duplications, translocations found in 3-5% of cases or small defects, such as different mutations responsible for 75% of cases of hemophilia A.
Fever in infants Measures to reduce the fever
Fever in infants
Measures to reduce the fever
"administer a medicine against fever, adjusted for age baby (acetaminophen or ibuprofen)
»room temperature where the child should not exceed 18-20 ° C.
"If the room is overheated, turn off the heater or other heat sources and open the window
»child naked and take her bath: water temperature should be 2 ° C lower than the baby's rectal temperature
"let the child in the water about 10 minutes
»Give your child plenty to drink because they can dehydrate very quickly
"If your child's temperature is above 40 ° C, called the emergency doctor
Fever may be accompanied by other signs of alarm. If your baby is fussy or on the contrary, apathetic, shows respiratory problems, vomiting, diarrhea or rash, call your doctor, even if the temperature is not large enough to become a fever.
Measures to reduce the fever
"administer a medicine against fever, adjusted for age baby (acetaminophen or ibuprofen)
»room temperature where the child should not exceed 18-20 ° C.
"If the room is overheated, turn off the heater or other heat sources and open the window
»child naked and take her bath: water temperature should be 2 ° C lower than the baby's rectal temperature
"let the child in the water about 10 minutes
»Give your child plenty to drink because they can dehydrate very quickly
"If your child's temperature is above 40 ° C, called the emergency doctor
Fever may be accompanied by other signs of alarm. If your baby is fussy or on the contrary, apathetic, shows respiratory problems, vomiting, diarrhea or rash, call your doctor, even if the temperature is not large enough to become a fever.
Fever in infants Causes of fever in infants
Fever in infants
Causes of fever in infants
Fever in infants may have numerous causes. The child may be dehydrated or too thick can be dressed in a relatively warm environment (the rule is that baby is dressed in a suit of clothes more than one adult).
But more often, fever is caused by an infection. The immune system detects a foreign body - a bacterium or virus - and reacts, causing elevated temperature. Some bacteria and viruses are sensitive to high temperatures and can easily be destroyed by the immune system. Thus, fever is a defense mechanism - indicate an infection and contribute to the fight against it.
Causes of fever in infants
Fever in infants may have numerous causes. The child may be dehydrated or too thick can be dressed in a relatively warm environment (the rule is that baby is dressed in a suit of clothes more than one adult).
But more often, fever is caused by an infection. The immune system detects a foreign body - a bacterium or virus - and reacts, causing elevated temperature. Some bacteria and viruses are sensitive to high temperatures and can easily be destroyed by the immune system. Thus, fever is a defense mechanism - indicate an infection and contribute to the fight against it.
Fever in infants
Fever in infants
* Introduction
* Causes of fever in infants
* Measures to reduce the fever
In the first months of baby's life, fever is a warning sign, because their immune system is still not fully matured and is able to fight effectively against infections.
Normal temperature in infants is 37 to 37.5 ° C and measured with a rectal thermometer medical thermometer or an ear. Since the normal temperature varies little from one baby to another, it is important to know who is the baby's normal temperature by measuring it a few times when not ill.
Over 38 ° C, it talks about the fever. Fever is common in infants, because they react to any infection by a sudden rise in temperature. This is a warning for young mothers, especially when it comes to their first child.
For parents it is important to measure the temperature correctly and the baby when he has a fever, to begin before the arrival of medical treatment.
* Introduction
* Causes of fever in infants
* Measures to reduce the fever
In the first months of baby's life, fever is a warning sign, because their immune system is still not fully matured and is able to fight effectively against infections.
Normal temperature in infants is 37 to 37.5 ° C and measured with a rectal thermometer medical thermometer or an ear. Since the normal temperature varies little from one baby to another, it is important to know who is the baby's normal temperature by measuring it a few times when not ill.
Over 38 ° C, it talks about the fever. Fever is common in infants, because they react to any infection by a sudden rise in temperature. This is a warning for young mothers, especially when it comes to their first child.
For parents it is important to measure the temperature correctly and the baby when he has a fever, to begin before the arrival of medical treatment.
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