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Truncus arteriosus is a congenital heart defect. At birth, the heart has only one blood vessel and one valve, instead of the usual two arteries and two valves. It is always associated with a ventricular septal defect, which is a hole in the partition that separates the two ventricles.
The single output vessel from the heart means that oxygenated blood is mixed with unoxygenated blood and then enters the lungs and the rest of the body. The arteries that supply the whole body and the lungs originate from this single vessel. The subsequent increased volume of blood that enters the lungs may result in congestive heart failure and lung damage.
The most common symptoms of truncus arteriosus are:
It is a rare heart disorder that affects fewer than 1 in 10,000 children and both genders are affected equally. It may be related to chromosome abnormalities such as DiGeorge syndrome, also known as 22q11 deletion.
Truncus arteriosus is diagnosed via echocardiogram, either prenatally or during the first few hours of life. This is usually sufficient to mean that further diagnosis and tests are not required.
It is repaired during prenatal surgery, during which the two circulations are separated and a conduit inserted into the pulmonary artery. The ventricular septal defect will also be closed.
Most children who undergo surgery recover and go on to develop normally.
In some cases further procedures may be necessary once the child reaches adulthood.
Complete transposition of the great arteries (TGA) is a congenital heart defect in which the aorta fully, or almost fully, exits the right ventricle (RV) and the pulmonary artery fully, or almost fully, exists the left ventricle (LV).
The key sign is cyanosis, which is when there is a blueish cast to the skin. Cyanosis is more intense in cases of complete transposition of the great arteries where there is no ventricular septal defect (VSD).
It tends to be diagnosed before birth through foetal ultrasound. Around 70% of cases are diagnosed in the first few months of foetal development.
Complete transposition of the great arteries can be associated with other anomalies such as ventricular septal defect, pulmonary stenosis or aortic arch hypoplasia. In these cases it is known as “complex complete transposition of the great arteries”.
The severity of any related defects must be investigated to determine the best type of surgery in each case.
An arterial switch, anatomical correction or the Jatene procedure is the most commonly used technique to treat complete transposition of the great arteries at the neonatal stage. Through this technique, the pulmonary artery is connected to the right ventricle and the aorta to the left ventricle. The coronary arteries are also transferred to the new aorta.
Most patients reach adulthood without needing further procedures and with a quality of life comparable to the normal population.
A congenital cyanotic heart defect is a congenital heart disorder in which deoxygenated blood bypasses the lungs and enters the circulatory system, or where there is a mixture of oxygenated and deoxygenated blood entering the system. It is caused by structural defects in the heart such as bidirectional shunting, or the incorrect position of the pulmonary artery or the aorta, or any condition that increases pulmonary vascular resistance. The result is the development of collateral circulation.
Children with this heart condition will have the following symptoms:
Tetralogy of Fallot makes up 10% of all congenital heart disorders and is considered the most common cyanotic heart disease. There are around 400 cases for every million births.
Diagnosis is confirmed via 2D echocardiogram.
Repair is carried out around six months old. If the baby suffers a episode before the defect has been corrected, treatment is started in the form of beta blockers to reduce lung spasms.
If very severe cyanotic episodes persist in babies under six months despite this treatment, then palliative surgery needs to be performed to take blood to the lungs. This surgery consists of making a connection between a systemic artery and the pulmonary arteries (a systemic-pulmonary fistula).
The definitive corrective surgery involves closing the ventricular septal defect with a patch and widening the outlet from the right ventricle.
In cases of severe pulmonary insufficiency there is progressive dilatation of the right ventricle in the long term. If this becomes excessive, it is necessary to replace the valve. Risk of lung valve replacement is around 20% after 25 years.
Unfortunately, there are currently no measures that can be taken to prevent this heart condition.
These syndromes are a group of diseases characterised by insufficient blood cell production (anaemia, neutropenia and thrombocytopenia), constitutional malformations and the risk of cancer.
They are usually diagnosed in childhood but there some cases diagnosed in adults. Specifically, these syndromes are: Fanconi anaemia, dyskeratosis congenita or selectively severe congenital neutropenia, Diamond-Blackfan anaemia, Diamond-Shwachman syndrome, and amegakaryocytic thrombocytopenia.
Malformations caused by inherited bone marrow failure syndromes affect the skin, bones, heart and digestive system as well as the urinary system, the central nervous system, and others. They may also affect the area around the bone marrow.
Besides this, they also increase the predisposition to cancers such as acute myeloid leukaemia, myelodysplastic syndrome and squamous carcinoma of the head, neck and reproductive organs.
These syndromes have important biological pathways in common related to cell growth and division such as the activation of the p53 gene that is responsible for halting the cell cycle, cell ageing and cell death. In addition, mutations have been identified in more than 80 genes.
Although this is treated in childhood, once the patient reaches adulthood monitoring is very important. Early diagnosis of these syndromes is essential to ensure the patient receives appropriate treatment. The aim is to minimise toxicity at the same time as allowing genetic counselling, and implementing strategies for cancer prevention and monitoring. This treatment should focus on treating the bone marrow failure, but also any constitutional malformations and extra-haematological manifestations, in addition to cancer treatment.
A multidisciplinary team of specialists with extensive experience of these diseases is necessary to ensure optimum patient care.
The first symptoms are related to a lack of blood cell production:
There are also signs related to malformations such as:
Estimated incidence rates of the different syndromes are:
The usual tests to detect inherited bone marrow failure are:
The following methods are used to treat these pathologies:
To prevent these diseases it is important to avoid smoking, alcohol, sun exposure, and to have a balanced diet. In addition, a cancer prevention programme should be followed with regular visits to the Cancer Prevention Unit and the Ear, Nose and Throat, Maxillofacial and Gynaecology departments.
The disease caused by the Zika virus is contracted by a bite from an infected mosquito, as in the case of dengue fever, chikungunya and yellow fever. It can also be spread through sexual intercourse, pregnant women may transmit it to their children, or through blood transfusions. In Europe there are no cases of infection by mosquito; all cases have been imported.
It is disease lasting a short time that can be overcome without complications or the need for admission to hospital. However, there is a relationship between this infection and some neurological disorders. In addition, pregnant women who are infected may give birth to babies with microcephaly.
The incubation period in humans is 3-12 days, up to 15 maximum. Although on many occasions there are no symptoms, when there are the disease is characterised by:
Since 2015, 71 countries have declared transmission of the Zika virus via mosquitoes. In addition, 13 more have stated that the disease has arrived by other means, generally through sexual contact.
In Europe, most cases have been imported from countries where it is endemic, mainly from Latin America but also from South East Asia. In Catalonia in December 2016, there were 150 registered infections, of which 32 were pregnant women.
Between the first seven to ten days of the disease, diagnosis is made using molecular biology techniques (RT-PCR) in blood and urine to detect the virus.
After this period, Zika disappears from the blood and is detected through antibodies in the serum.
There is no specific treatment for this disease. Symptoms generally disappear between three and seven days after infection. They are therefore lessened with analgesics and antipyretics.
There is currently no vaccine for this virus. For this reason, prevention is based on avoiding mosquito bites in countries where it is endemic, as well as using protection during sexual intercourse.
In the case of Catalonia, the risk is associated with the arrival of travellers from countries where it is endemic. Here there is a screening programme for pregnant women and their partners; they are a sensitive group as the virus may be passed to the foetus.
Pulmonary atresia with ventricular septal defect is a rare heart condition characterised by a lack of connection between the right ventricle and the pulmonary arteries.
This is a rare congenital heart defect characterised by no connection between the right ventricle and the pulmonary arteries. It is an extreme type of Tetralogy of Fallot in which blood enters the lungs to be oxygenated by bypassing the heart.
Blood can reach the lungs via the pulmonary arteries themselves, which are not connected to the heart, or via the collateral arteries, which originate from the thoracic aorta and directly supply the lung. There are significant anatomical differences between vessels which must be studied in each individual child.
This condition is very heterogeneous, which creates the variability seen in the pulmonary arteries. Two groups can be distinguished:
The prognosis of this disease depends on the growth of the pulmonary arteries to be able to surgically repair the condition.
It is a rare congenital heart condition which makes up 1-2% of all congenital heart defects.
In most cases, diagnosis is via foetal echocardiogram. This ultrasound will show the lack of connection between the heart and the pulmonary arteries, as well as the presence of VSD. Through this test the size and position of the pulmonary arteries can also be measured.
When a child is born, it has a certain quantity of oxygen, known as “saturation”, in its blood which is around 80-90% of the normal level, although this is enough for the child to develop normally.
Childhood dystonia is progressive and debilitating, but it can be prevented with an early diagnosis and the use of specific therapies that target the genetic defect identified. Currently, several different treatments are used.
Levodopa trial
In children with isolated dystonia, a one to three-month long trial with levodopa must always be carried out. This medication can completely reverse symptoms when the dystonia is caused by a defect in dopamine synthesis. This is called dopa-responsive dystonia.
Such defective dopamine synthesis can be caused by alterations in the genes and proteins that regulate dopamine metabolism. This neurotransmitter is essential in learning, behaviour, and motor function.
Treatment with botulinum toxin
Botulinum toxin is effective at controlling focal dystonia, which affects a muscle group carrying out a specific action, such as, for example, writer’s cramp, or dystonia triggered by walking, treatment for which consists of correcting the abnormal foot or leg position when walking.
In children with generalised dystonia, botulinum toxin can also relieve pain in muscle groups that are particularly affecting the patient’s quality of life.
Treatment with various pharmaceuticals
To treat generalised dystonia in children, different drugs are administered to reduce the tremors, muscle tone, and painful spasms. These include benzodiazepines, GABA antagonists (baclofen), anticholinergics, and α-2 adrenergic receptor agonists. In some patients with paroxysmal dystonia, which is characterised by brief, repetitive involuntary movements at night, anti-epileptics are prescribed.
Intrathecal baclofen pump
When oral medications are not enough, there is a surgical option: the intrathecal baclofen pump. This internal device administers the medication baclofen through a catheter placed in the epidural space (which contains the liquid that surrounds the spinal cord). This helps control generalised dystonia, reducing pain, muscle tone, and spasms and thus improving patients’ quality of life.
This device is used to treat secondary dystonia or dystonia associated with other neurological problems (whether neurometabolic, neurodegenerative, or acquired through brain damage at birth).
Deep brain stimulation or pallidal stimulation
This consists of placing two electrodes in the pallidal nuclei of the brain via a stereotactic procedure, to improve the patient’s motor function and quality of life. Patients who are candidates for pallidal stimulation are those with primary dystonia who have no structural brain lesions in the central nervous system.
Together with haemodialysis, peritoneal dialysis is an extra-renal filtration procedure. Kidney failure is treated with dialysis, a word that means “pass through”, and which uses the patient’s peritoneum as a filter. The peritoneum is the membrane that lines the abdominal cavity and it has a large surface area of around one square metre. This peritoneal membrane can filter out substances that need to be removed from the body (urea, potassium, phosphorus and many others) when filled with a glucose-rich dialysis fluid that encourages waste to be passed from the patient’s blood into it.
The procedure is as follows: a catheter is inserted into the navel for introducing the dialysis fluid. This fluid is left in the peritoneal cavity for some time and then exchanged for new fluid. This is repeated several times.
Peritoneal dialysis can be performed at the patient’s home and also at night, which is an important factor to maintain the patient's quality of life.
Possible complications of peritoneal dialysis are peritonitis or infection of the dialysis fluid which can lead to infection and inflammation of the peritoneum. Treatment with antibiotics is effective for this complication.
Haemodialysis is usually considered to an intermediary step between advanced kidney failure and a kidney transplant.
Haemodialysis is an extra-renal filtration procedure that replaces kidney function using an external system. It acts as a filter for the patient's blood by connecting to the patient’s circulatory system via a catheter or by being directly inserted into the vein, usually in the arm. In other cases, an arteriovenous fistula may be created, connecting an artery to a vein beneath the skin on the arm. When an artery is connected to a vein, pressure in the vein increases, strengthening the vein walls. This stronger vein is able to withstand the needles used in haemodialysis and greater blood flow is achieved.
People with kidney failure starting a haemodialysis programme typically have less than 10% of normal kidney function. Above this figure, haemodialysis is usually not necessary.
Haemodialysis must be performed regularly in four-hour sessions, usually three times a week, although the duration and frequency will depend on the patient and their circumstances.
Haemodialysis is based on biophysics in the sense that the blood passes through a filter and exchanges substances with the fluid on the other side of the filter, which is circulated by a machine. The exchange gets rids of the urea, potassium, phosphorus and other waste substances that build up due to lack of kidney function. These substances partly pass through the membrane by themselves, as there are different concentrations of the substances and they tend to equalise, and is also due to the changes in pressure exerted by the haemodialysis machine.
Possible complications of haemodialysis are the infection of the catheter or being unable to find a viable vascular access site in patients who have had dialysis for many years.
Haemodialysis may continue for years, although it is usually an intermediate step between kidney failure and transplant.
There are four basic parts to treating renal insufficiency.
Controlling arterial pressure, if it is high; levels of urea; the balance of mineral salts (sodium, potassium, calcium, phosphorus, magnesium); acidity and anaemia. Analytical testing provides a lot of information which enables the origin and severity of the kidney disease to be established.
A kidney biopsy allows a microscopic study that is often essential. Genetic testing also provides very important information.
There are three different levels of treatment:
a) medical, with the use of medication or hormones to substitute the alterations mentioned. A diet that creates little urea or that contains low levels of potassium, drugs to control excess or lack of sodium, potassium, calcium, phosphorus, magnesium or acidity. And medication to treat anaemia.
b) extrarenal purification methods: haemodialysis (passing the blood through an external circuit to purify it and filter out toxic substances using a suitable filter), and peritoneal dialysis, during which a solution is circulated inside the patient's peritoneal cavity and is then extracted, taking the toxic substances usually expelled through urine with it.
c) kidney transplant from a living or deceased donor. In this instance, the new kidney takes over the functions of the diseased kidney. How long a kidney graft lasts varies and relies on controlling episodes of organ rejection that may occur after transplant. A young patient with kidney insufficiency may require more than one kidney transplant over their lifetime, although the useful life of these grafts is increasing day by day thanks to new immunosuppressant drugs.
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